A subclass of ACIDIC GLYCOSPHINGOLIPIDS. They contain one or more sialic acid (N-ACETYLNEURAMINIC ACID) residues. Using the Svennerholm system of abbrevations, gangliosides are designated G for ganglioside, plus subscript M, D, or T for mono-, di-, or trisialo, respectively, the subscript letter being followed by a subscript arabic numeral to indicated sequence of migration in thin-layer chromatograms. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1997)
A ganglioside present in abnormally large amounts in the brain and liver due to a deficient biosynthetic enzyme, G(M3):UDP-N-acetylgalactosaminyltransferase. Deficiency of this enzyme prevents the formation of G(M2) ganglioside from G(M3) ganglioside and is the cause of an anabolic sphingolipidosis.
A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis.
Chromatography on thin layers of adsorbents rather than in columns. The adsorbent can be alumina, silica gel, silicates, charcoals, or cellulose. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
A glycosphingolipid that accumulates due to a deficiency of hexosaminidase A or B (BETA-N-ACETYLHEXOSAMINIDASES), or GM2 activator protein, resulting in GANGLIOSIDOSES, heredity metabolic disorders that include TAY-SACHS DISEASE and SANDHOFF DISEASE.
Lipids containing at least one monosaccharide residue and either a sphingoid or a ceramide (CERAMIDES). They are subdivided into NEUTRAL GLYCOSPHINGOLIPIDS comprising monoglycosyl- and oligoglycosylsphingoids and monoglycosyl- and oligoglycosylceramides; and ACIDIC GLYCOSPHINGOLIPIDS which comprises sialosylglycosylsphingolipids (GANGLIOSIDES); SULFOGLYCOSPHINGOLIPIDS (formerly known as sulfatides), glycuronoglycosphingolipids, and phospho- and phosphonoglycosphingolipids. (From IUPAC's webpage)
A group of naturally occurring N-and O-acyl derivatives of the deoxyamino sugar neuraminic acid. They are ubiquitously distributed in many tissues.
The sequence of carbohydrates within POLYSACCHARIDES; GLYCOPROTEINS; and GLYCOLIPIDS.
Neuraminic acids are a family of nine-carbon sugars (sialic acids) that are commonly found as terminal residues on glycoproteins and gangliosides in animal tissues, playing crucial roles in various biological processes including cell recognition, inflammation, and bacterial/viral infectivity.
An enzyme that catalyzes the hydrolysis of alpha-2,3, alpha-2,6-, and alpha-2,8-glycosidic linkages (at a decreasing rate, respectively) of terminal sialic residues in oligosaccharides, glycoproteins, glycolipids, colominic acid, and synthetic substrate. (From Enzyme Nomenclature, 1992)
A group of enzymes with the general formula CMP-N-acetylneuraminate:acceptor N-acetylneuraminyl transferase. They catalyze the transfer of N-acetylneuraminic acid from CMP-N-acetylneuraminic acid to an acceptor, which is usually the terminal sugar residue of an oligosaccharide, a glycoprotein, or a glycolipid. EC 2.4.99.-.
An N-acyl derivative of neuraminic acid. N-acetylneuraminic acid occurs in many polysaccharides, glycoproteins, and glycolipids in animals and bacteria. (From Dorland, 28th ed, p1518)
Enzymes that catalyze the transfer of N-acetylgalactosamine from a nucleoside diphosphate N-acetylgalactosamine to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.
Any compound containing one or more monosaccharide residues bound by a glycosidic linkage to a hydrophobic moiety such as an acylglycerol (see GLYCERIDES), a sphingoid, a ceramide (CERAMIDES) (N-acylsphingoid) or a prenyl phosphate. (From IUPAC's webpage)
GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups.
A mass spectrometric technique that is used for the analysis of a wide range of biomolecules, such as glycoalkaloids, glycoproteins, polysaccharides, and peptides. Positive and negative fast atom bombardment spectra are recorded on a mass spectrometer fitted with an atom gun with xenon as the customary beam. The mass spectra obtained contain molecular weight recognition as well as sequence information.
Changes in the amounts of various chemicals (neurotransmitters, receptors, enzymes, and other metabolites) specific to the area of the central nervous system contained within the head. These are monitored over time, during sensory stimulation, or under different disease states.
Neutral glycosphingolipids that contain a monosaccharide, normally glucose or galactose, in 1-ortho-beta-glycosidic linkage with the primary alcohol of an N-acyl sphingoid (ceramide). In plants the monosaccharide is normally glucose and the sphingoid usually phytosphingosine. In animals, the monosaccharide is usually galactose, though this may vary with the tissue and the sphingoid is usually sphingosine or dihydrosphingosine. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1st ed)
The characteristic 3-dimensional shape of a carbohydrate.
An acute inflammatory autoimmune neuritis caused by T cell- mediated cellular immune response directed towards peripheral myelin. Demyelination occurs in peripheral nerves and nerve roots. The process is often preceded by a viral or bacterial infection, surgery, immunization, lymphoma, or exposure to toxins. Common clinical manifestations include progressive weakness, loss of sensation, and loss of deep tendon reflexes. Weakness of respiratory muscles and autonomic dysfunction may occur. (From Adams et al., Principles of Neurology, 6th ed, pp1312-1314)
Glycosphingolipids containing N-acetylglucosamine (paragloboside) or N-acetylgalactosamine (globoside). Globoside is the P antigen on erythrocytes and paragloboside is an intermediate in the biosynthesis of erythrocyte blood group ABH and P 1 glycosphingolipid antigens. The accumulation of globoside in tissue, due to a defect in hexosaminidases A and B, is the cause of Sandhoff disease.
Members of the class of neutral glycosphingolipids. They are the basic units of SPHINGOLIPIDS. They are sphingoids attached via their amino groups to a long chain fatty acyl group. They abnormally accumulate in FABRY DISEASE.
A group of autosomal recessive lysosomal storage disorders marked by the accumulation of GANGLIOSIDES. They are caused by impaired enzymes or defective cofactors required for normal ganglioside degradation in the LYSOSOMES. Gangliosidoses are classified by the specific ganglioside accumulated in the defective degradation pathway.
Protein synthesized by CLOSTRIDIUM TETANI as a single chain of ~150 kDa with 35% sequence identity to BOTULINUM TOXIN that is cleaved to a light and a heavy chain that are linked by a single disulfide bond. Tetanolysin is the hemolytic and tetanospasmin is the neurotoxic principle. The toxin causes disruption of the inhibitory mechanisms of the CNS, thus permitting uncontrolled nervous activity, leading to fatal CONVULSIONS.
A group of recessively inherited diseases characterized by the intralysosomal accumulation of G(M2) GANGLIOSIDE in the neuronal cells. Subtypes include mutations of enzymes in the BETA-N-ACETYLHEXOSAMINIDASES system or G(M2) ACTIVATOR PROTEIN leading to disruption of normal degradation of GANGLIOSIDES, a subclass of ACIDIC GLYCOSPHINGOLIPIDS.
Conditions characterized by abnormal lipid deposition due to disturbance in lipid metabolism, such as hereditary diseases involving lysosomal enzymes required for lipid breakdown. They are classified either by the enzyme defect or by the type of lipid involved.
Diseases characterized by injury or dysfunction involving multiple peripheral nerves and nerve roots. The process may primarily affect myelin or nerve axons. Two of the more common demyelinating forms are acute inflammatory polyradiculopathy (GUILLAIN-BARRE SYNDROME) and POLYRADICULONEUROPATHY, CHRONIC INFLAMMATORY DEMYELINATING. Polyradiculoneuritis refers to inflammation of multiple peripheral nerves and spinal nerve roots.
An ENTEROTOXIN from VIBRIO CHOLERAE. It consists of two major protomers, the heavy (H) or A subunit and the B protomer which consists of 5 light (L) or B subunits. The catalytic A subunit is proteolytically cleaved into fragments A1 and A2. The A1 fragment is a MONO(ADP-RIBOSE) TRANSFERASE. The B protomer binds cholera toxin to intestinal epithelial cells, and facilitates the uptake of the A1 fragment. The A1 catalyzed transfer of ADP-RIBOSE to the alpha subunits of heterotrimeric G PROTEINS activates the production of CYCLIC AMP. Increased levels of cyclic AMP are thought to modulate release of fluid and electrolytes from intestinal crypt cells.
The largest class of organic compounds, including STARCH; GLYCOGEN; CELLULOSE; POLYSACCHARIDES; and simple MONOSACCHARIDES. Carbohydrates are composed of carbon, hydrogen, and oxygen in a ratio of Cn(H2O)n.
Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.
A variant of the GUILLAIN-BARRE SYNDROME characterized by the acute onset of oculomotor dysfunction, ataxia, and loss of deep tendon reflexes with relative sparing of strength in the extremities and trunk. The ataxia is produced by peripheral sensory nerve dysfunction and not by cerebellar injury. Facial weakness and sensory loss may also occur. The process is mediated by autoantibodies directed against a component of myelin found in peripheral nerves. (Adams et al., Principles of Neurology, 6th ed, p1313; Neurology 1987 Sep;37(9):1493-8)
Cerebrosides which contain as their polar head group a glucose moiety bound in glycosidic linkage to the hydroxyl group of ceramides. Their accumulation in tissue, due to a defect in beta-glucosidase, is the cause of Gaucher's disease.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
An amino alcohol with a long unsaturated hydrocarbon chain. Sphingosine and its derivative sphinganine are the major bases of the sphingolipids in mammals. (Dorland, 28th ed)
Carbohydrates covalently linked to a nonsugar moiety (lipids or proteins). The major glycoconjugates are glycoproteins, glycopeptides, peptidoglycans, glycolipids, and lipopolysaccharides. (From Biochemical Nomenclature and Related Documents, 2d ed; From Principles of Biochemistry, 2d ed)
The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.
The genetic complement of an organism, including all of its GENES, as represented in its DNA, or in some cases, its RNA.
The addition of descriptive information about the function or structure of a molecular sequence to its MOLECULAR SEQUENCE DATA record.
Databases devoted to knowledge about specific genes and gene products.
A multistage process that includes cloning, physical mapping, subcloning, determination of the DNA SEQUENCE, and information analysis.
The systematic study of the complete DNA sequences (GENOME) of organisms.

The role of homophilic binding in anti-tumor antibody R24 recognition of molecular surfaces. Demonstration of an intermolecular beta-sheet interaction between vh domains. (1/1980)

The murine antibody R24 and mouse-human Fv-IgG1(kappa) chimeric antibody chR24 are specific for the cell-surface tumor antigen disialoganglioside GD3. X-ray diffraction and surface plasmon resonance experiments have been employed to study the mechanism of "homophilic binding," in which molecules of R24 recognize and bind to other molecules of R24 though their heavy chain variable domains. R24 exhibits strong binding to liposomes containing disialoganglioside GD3; however, the kinetics are unusual in that saturation of binding is not observed. The binding of chR24 to GD3-bearing liposomes is significantly weaker, suggesting that cooperative interactions involving antibody constant regions contribute to R24 binding of membrane-bound GD3. The crystal structures of the Fabs from R24 and chR24 reveal the mechanism for homophilic binding and confirm that the homophilic and antigen-binding idiotopes are distinct. The homophilic binding idiotope is formed largely by an anti-parallel beta-sheet dimerization between the H2 complementarity determining region (CDR) loops of two Fabs, while the antigen-binding idiotope is a pocket formed by the three CDR loops on the heavy chain. The formation of homophilic dimers requires the presence of a canonical conformation for the H2 CDR in conjunction with participation of side chains. The relative positions of the homophilic and antigen-binding sites allows for a lattice of GD3-specific antibodies to be constructed, which is stabilized by the presence of the cell membrane. This model provides for the selective recognition by R24 of cells that overexpress GD3 on the cell surface.  (+info)

Partial purification and properties of porcine thymus lactosylceramide beta-galactosidase. (2/1980)

Porcine thymus lactosylceramide beta-galactosidase was purified by a simple procedure. In the final step of isoelectric focusing the enzyme was separated into two peaks of pI 6.3 (peak I) and 7.0 (peak II), which showed 3,600- and 4,000-fold enhancement of lactosylceramide-hydrolysing activity, respectively. The two peaks had identical mobility on polyacrylamide gel electrophoresis. The apparent molecular weight was 34,000. Neither monosialoganglioside (GM1) nor galactosylceramide was hydrolysed by the purified enzyme fractions. The optimal pH was at 4.6, and sodium taurocholate was essential for the reaction. The apparent Km was 2.3 x 10-5 M. The reaction was stimulated by sodium chloride and linoleic acid, while it was strongly inhibited by Triton X-100 and bovine serum albumin. Galactosylceramide, p-nitrophenyl beta-galactoside, and p-nitrophenol were weak inhibitors. No effects of GM1 and galactose were observed on the hydrolysis of lactosylceramide.  (+info)

Gangliosides of human kidney. (3/1980)

Five gangliosides isolated from human kidney have been characterized. The two main fractions were shown to be typical extraneural gangliosides in having lactose as their neutral carbohydrate moiety. Their structures were identified as: AcNeu(alpha2-3)Gal(beta1-4)Glc(beta1-1)Cer and AcNeu(alpha2-8)AcNeu(alpha2-3)Gal(beta1-4)Glc(beta1-1)Cer. The two main hexosamine-containing gangliosides are structurally related to human blood group substances of glycosphingolipid nature. The following structures are postulated: AcNeu(alpha2-3)Gal(beta1-4)GlcNAc(beta1-3)Gal(beta1-4)Glc(beta1-1)Cer and AcNeu(alpha2-3)Gal(beta1-4)[Fuc(alpha1-3)]GlcNAc(beta1-3)Gal(beta1-4)Glc(beta1-1) Cer. The third hexosamine-containing ganglioside belongs to a different series of glycolipids and was shown to have the structure of a major ganglioside of human brain: AcNeu(alpha2-3)Gal(beta1-3)GalNAc(beta1-4)[AcNeu(alpha2-3)]Gal(beta1-4)Glc(beta1- 1)Cer. The fatty acid structure of different gangliosides was shown to resemble that of neutral glycolipids of human kidney with the nonhydroxy acids C16:0, C22:0, and C24:0 as major components.  (+info)

Lipolytic action of cholera toxin on fat cells. Re-examination of the concept implicating GM1 ganglioside as the native membrane receptor. (4/1980)

The possible role of galactosyl-N-acetylgalactosaminyl-[N-acetylneuraminyl]-galactosylglucosylceramide (GM1) ganglioside in the lipolytic activity of cholera toxin on isolated fat cells has been examined. Analyses of the ganglioside content and composition of intact fat cells, their membranous ghosts, and the total particulate fraction of these cells indicate that N-acetylneuraminylgalactosylglucosylceramide (GM3) represents the major ganglioside, with substantial amounts of N-acetylgalactosaminyl-[N-acetylneuraminyl]-galactosylglucosylceramide (GM2) and smaller amounts of other higher homologues also present. Native GM1 was not detected in any of these preparations. Examination of the relative capacities of various exogenously added radiolabeled sphingolipids to bind to the cells indicated that GM2 and glucosylsphingosine were accumulated by the cells to extents comparable to GM1. Galactosylsphingosine and sulfatide also exhibited significant, although lesser, binding affinities for the cells. The adipocytes appeared to nonspecifically bind exogenously added GM1; saturation of binding sites for GM1 could not be observed up to the highest concentration tested (2 X 10(-4) M), wherein about 7 X 10(9) molecules were associated with the cells. Essentially all of this exogenously added GM1 was found bound to the plasma membrane "ghost" fraction. Investigation of the biological responses of the cells confirmed their sensitivities to both cholera toxin and epinephrine-stimulated lipolysis, as well as the lag period displayed during the toxin's action. While we could confirm that the toxin's lipolytic activity can be enhanced by prior treatment of the fat cells with GM1, several of the observed characteristics of this phenomenon differ from earlier reported findings. Accordingly, added GM1 was able to enhance only the subsequent rate, but not the extent, of toxin-stimulated glycerol release (lipolysis) from the cells. We also were unable to confirm the ability of GM1 to enhance the toxin's activity at either saturating or at low toxin concentrations. The limited ability of added GM1 to enhance the toxin's activity appeared in a unique bell-shaped dose-response manner. The inability of high levels of GM1 to stimulate a dose of toxin that was ineffective on native cells suggests that the earlier reported ability of crude brain gangliosides to accomplish this was due to some component other than GM1 in the crude extract. While several glycosphingolipids and some other carbohydrate-containing substances that were tested lacked the ability to mimic the enhancing effect of GM1, 4-methylumbelliferyl-beta-D-galactoside exhibited an effect similar to, although less pronounced than, that of GM1. The findings in these studies are unable to lend support to the earlier hypothesis that (a) GM1 is cholera toxin's naturally occurring membrane receptor on native fat cells, and (b) the ability of exogenously added GM1 to enhance the toxin's lipolytic activity represents the specific creation of additional natural receptors on adipocytes...  (+info)

Atherosclerotic aortic gangliosides enhance integrin-mediated platelet adhesion to collagen. (5/1980)

Gangliosides, sialic acid-containing glycosphingolipids, accumulate in atherosclerotic vessels. Their role in the pathogenesis of atherosclerosis is unknown. Gangliosides isolated from tumor cells promote collagen-stimulated platelet aggregation and ATP secretion and enhance platelet adhesion to immobilized collagen. These activities are all mediated by ganglioside effects on the platelet integrin collagen receptor alpha2beta1. Therefore, we hypothesized that gangliosides isolated from atherosclerotic plaques would enhance platelet adhesion to immobilized collagen, a major component of the subendothelial matrix of blood vessels. Furthermore, we questioned whether this effect of atherosclerotic gangliosides might play a role in the pathogenesis of atherosclerosis. To test this hypothesis, we isolated the gangliosides from postmortem aortas of patients with extensive atherosclerotic disease and examined their effects on platelet adhesion. Samples of aortic tissue taken from areas involved with atherosclerotic plaque demonstrated accumulation of gangliosides (64.9+/-6.5 nmol/g wet weight) compared with gangliosides isolated from control normal aortic tissue taken from children who died of noncardiac causes (NAGs; 21.1+/-6.4 nmol/g wet weight). Interestingly, samples of tissue taken from diseased aortas but from areas not involved with gross plaque formation also demonstrated ganglioside accumulation (47.6+/-12.8 nmol/g wet weight). Next, the activity of each of these gangliosides on platelet adhesion to immobilized type I collagen was studied. Atherosclerotic aortic gangliosides (AAGs) as well as those isolated from grossly unaffected areas of the same aorta (UAGs) both increased platelet adhesion compared with control NAGs (OD570, 0. 37+/-0.11 and 0.29+/-0.14 versus 0.16+/-0.07, respectively; P<0.01 and P<0.05, respectively). These OD570 values corresponded to 9x10(5), 8x10(4), and 6x10(3) platelets per well after preincubation with 5 micromol/L AAG, UAG, and NAG, respectively. Increased adhesion was observed after preincubation with as little as 0.5 micromol/L AAG, and maximal adhesion was seen at 2.5 micromol/L, with a plateau extending to the highest concentration tested, 10 micromol/L. The effect of AAGs on platelet adhesion to collagen was abrogated by incubation of treated platelets with F-17 anti-alpha2 monoclonal antibody (OD570, 0.13+/-0.02). Finally, the effects of the major individual gangliosides isolated from atherosclerotic tissues, GM3 and GD3, were tested. GM3 increased adhesion to collagen (OD570, 0.415+/-0.06) as did GD3 (0.31+/-0.08). Similar to that of AAGs, the effect of both molecules was blocked by F-17 (0. 09+/-0.04 and 0.13+/-0.06, respectively). These experiments demonstrate that accumulated atherosclerotic gangliosides promote platelet adhesion to collagen, the major component of the subendothelial matrix. Furthermore, this activity is mediated by an effect of the gangliosides on the collagen-binding integrin alpha2beta1. This activity may provide a mechanism for the development of platelet thrombi at sites where atherosclerotic gangliosides accumulate and help to explain the role of platelets in the process of atherosclerotic disease progression.  (+info)

Antiganglioside antibodies in Guillain-Barre syndrome after a recent cytomegalovirus infection. (6/1980)

OBJECTIVE: To study the association between anti-ganglioside antibody responses and Guillan-Barre syndrome (GBS) after a recent cytomegalovirus (CMV) infection. METHODS: Enzyme linked immunosorbant assay (ELISA) was undertaken on serum samples from 14 patients with GBS with recent cytomegalovirus (CMV) infection (CMV+GBS) and 12 without (CMV-GBS), 17 patients with other neurological diseases (OND), 11 patients with a recent CMV infection but without neurological involvement, 11 patients with recent Epstein-Barr virus (EBV) infection but without neurological involvement, and 20 normal control (NC) subjects. RESULTS: IgM antibodies were found at 1:100 serum dilution to gangliosides GM2 (six of 14 patients), GM1 (four of 14), GD1a (three of 14) and GD1b (two of 14) in the serum samples of the CMV+GBS patients, but not in those of any of the CMV-GBS patients. IgM antibodies were also found to gangliosides GM1, GD1a, and GD1b in one of 11 OND patients, to ganglioside GM1 in one of 11 non- neurological CMV patients, and to ganglioside GD1b in one of 20 NC subjects. Some patients with EBV infection had IgM antibodies to gangliosides GM1 (five of 11), GM2 (three of 11), and GD1a (two of 11). However, the antibodies to ganglioside GM2 had a low titre, none being positive at 1:200 dilution, whereas five of the CMV+GBS serum samples remained positive at this dilution. CONCLUSION: Antibodies to ganglioside GM2 are often associated with GBS after CMV infection, but their relevance is not known. It is unlikely that CMV infection and anti-ganglioside GM2 antibodies are solely responsible and an additional factor is required to elicit GBS.  (+info)

The distribution of ganglioside-like moieties in peripheral nerves. (7/1980)

GM1 ganglioside has been implicated as a target of immune attack in some diseases of the peripheral nervous system. Anti-GM1 ganglioside antibodies are associated with certain acquired immune-mediated neuropathies. It is not clear how anti-GM1 antibodies cause nerve dysfunction and injury; however, sodium and/or potassium ion channel dysfunction at the node of Ranvier has been implicated. To gain insight into the pathogenesis of these neuropathies, we examined the distribution of GM1 ganglioside and Gal(beta1-3)GalNAc moieties in nerve fibres and their relationship to voltage-gated sodium and potassium (Kv1.1, 1.5) channels at the nodes of Ranvier in peripheral nerves from human, rat and dystrophic mice. Gal(beta1-3)GalNAc moieties were localized via the binding of cholera toxin and peanut agglutinin. As a control for the specificity of these findings, we compared the distribution of GM1 moieties to that of the ganglioside GT1b. Our study provides definitive evidence for the presence of Gal(beta1-3)GalNAc bearing moieties on the axolemmal surface of mature myelinated fibres and on Schwann cells. Gal(beta1-3)GalNAc binding sites did not have an obligatory co-localization with voltage-gated sodium channels or the potassium ion channels Kv1.1 and Kv1.5 and are thus not likely carried by these ion channels. In contrast with Gal(beta1-3)GalNAc, GT1b-like moieties are restricted to the axolemma.  (+info)

De-N-acetyl-gangliosides in humans: unusual subcellular distribution of a novel tumor antigen. (8/1980)

The disialoganglioside GD3 is a major antigen in human melanomas that can undergo 9-O-acetylation of the outer sialic acid (giving 9-OAc-GD3). Monoclonal antibody SGR37 detects a different modification of the GD3, de-N-acetylation of the 5-N-acetyl group (giving de-N-Ac-GD3). We found that conventional immunohistochemistry of the SGR37 antigen is limited by a reduction in reactivity upon fixation with aldehydes (which presumably react with the free amino group) or with organic reagents (which can extract glycolipids). We optimized conditions for detection of this antigen in unfixed frozen tissue sections and studied its distribution in human tissues and tumors. It is expressed at low levels in a few blood vessels, infiltrating mononuclear cells in the skin and colon, and at moderate levels in skin melanocytes. In contrast, the antigen accumulates at high levels in many melanomas and in some lymphomas but not in carcinomas. In positive melanomas, expression is sometimes more intense and widespread than that of GD3. Both 9-O-acetylation and de-N-acetylation of GD3 seem to occur after its initial biosynthesis. Isotype-matched antibodies against GD3, 9-O-acetyl-GD3 and de-N-acetyl-GD3 were used to compare their subcellular localization and trafficking. 9-O-acetyl-GD3 colocalizes with GD3 predominantly on the cell surface and partly in lysosomal compartments. In contrast, de-N-acetyl-GD3 has a diffuse intracellular location. Adsorptive endocytosis of antibodies indicates that whereas GD3 remains predominantly on the cell surface, de-N-acetyl-GD3 is efficiently internalized into a compartment that is distinct from lysosomes. Rounding up of melanoma cells occurring during growth in culture is associated with relocation of the internal pool of de-N-acetyl-GD3 to the cell surface. Thus, a minor modification of the polar head group of a tumor-associated glycosphingolipid can markedly affect the subcellular localization and trafficking of the whole molecule. The high levels of the SGR37 antigen in melanomas and lymphomas, its selective endocytosis from the cell surface, and its relocation to the cell surface of rounded up cells suggest potential uses in diagnostic or therapeutic approaches to these diseases.  (+info)

Gangliosides are a type of complex lipid molecule known as sialic acid-containing glycosphingolipids. They are predominantly found in the outer leaflet of the cell membrane, particularly in the nervous system. Gangliosides play crucial roles in various biological processes, including cell recognition, signal transduction, and cell adhesion. They are especially abundant in the ganglia (nerve cell clusters) of the peripheral and central nervous systems, hence their name.

Gangliosides consist of a hydrophobic ceramide portion and a hydrophilic oligosaccharide chain that contains one or more sialic acid residues. The composition and structure of these oligosaccharide chains can vary significantly among different gangliosides, leading to the classification of various subtypes, such as GM1, GD1a, GD1b, GT1b, and GQ1b.

Abnormalities in ganglioside metabolism or expression have been implicated in several neurological disorders, including Parkinson's disease, Alzheimer's disease, and various lysosomal storage diseases like Tay-Sachs and Gaucher's diseases. Additionally, certain bacterial toxins, such as botulinum neurotoxin and tetanus toxin, target gangliosides to gain entry into neuronal cells, causing their toxic effects.

Thin-layer chromatography (TLC) is a type of chromatography used to separate, identify, and quantify the components of a mixture. In TLC, the sample is applied as a small spot onto a thin layer of adsorbent material, such as silica gel or alumina, which is coated on a flat, rigid support like a glass plate. The plate is then placed in a developing chamber containing a mobile phase, typically a mixture of solvents.

As the mobile phase moves up the plate by capillary action, it interacts with the stationary phase and the components of the sample. Different components of the mixture travel at different rates due to their varying interactions with the stationary and mobile phases, resulting in distinct spots on the plate. The distance each component travels can be measured and compared to known standards to identify and quantify the components of the mixture.

TLC is a simple, rapid, and cost-effective technique that is widely used in various fields, including forensics, pharmaceuticals, and research laboratories. It allows for the separation and analysis of complex mixtures with high resolution and sensitivity, making it an essential tool in many analytical applications.

Glycosphingolipids are a type of complex lipid molecule found in animal cell membranes, particularly in the outer leaflet of the plasma membrane. They consist of a hydrophobic ceramide backbone, which is composed of sphingosine and fatty acids, linked to one or more hydrophilic sugar residues, such as glucose or galactose.

Glycosphingolipids can be further classified into two main groups: neutral glycosphingolipids (which include cerebrosides and gangliosides) and acidic glycosphingolipids (which are primarily gangliosides). Glycosphingolipids play important roles in various cellular processes, including cell recognition, signal transduction, and cell adhesion.

Abnormalities in the metabolism or structure of glycosphingolipids have been implicated in several diseases, such as lysosomal storage disorders (e.g., Gaucher's disease, Fabry's disease) and certain types of cancer (e.g., ganglioside-expressing neuroblastoma).

Sialic acids are a family of nine-carbon sugars that are commonly found on the outermost surface of many cell types, particularly on the glycoconjugates of mucins in various secretions and on the glycoproteins and glycolipids of cell membranes. They play important roles in a variety of biological processes, including cell recognition, immune response, and viral and bacterial infectivity. Sialic acids can exist in different forms, with N-acetylneuraminic acid being the most common one in humans.

A "carbohydrate sequence" refers to the specific arrangement or order of monosaccharides (simple sugars) that make up a carbohydrate molecule, such as a polysaccharide or an oligosaccharide. Carbohydrates are often composed of repeating units of monosaccharides, and the sequence in which these units are arranged can have important implications for the function and properties of the carbohydrate.

For example, in glycoproteins (proteins that contain carbohydrate chains), the specific carbohydrate sequence can affect how the protein is processed and targeted within the cell, as well as its stability and activity. Similarly, in complex carbohydrates like starch or cellulose, the sequence of glucose units can determine whether the molecule is branched or unbranched, which can have implications for its digestibility and other properties.

Therefore, understanding the carbohydrate sequence is an important aspect of studying carbohydrate structure and function in biology and medicine.

Neuraminic acids, also known as sialic acids, are a family of nine-carbon sugars that are commonly found on the outermost layer of many cell surfaces in animals. They play important roles in various biological processes, such as cell recognition, immune response, and viral and bacterial infection. Neuraminic acids can exist in several forms, with N-acetylneuraminic acid (NANA) being the most common one in mammals. They are often found attached to other sugars to form complex carbohydrates called glycoconjugates, which are involved in many cellular functions and interactions.

Neuraminidase is an enzyme that occurs on the surface of influenza viruses. It plays a crucial role in the life cycle of the virus by helping it to infect host cells and to spread from cell to cell within the body. Neuraminidase works by cleaving sialic acid residues from glycoproteins, allowing the virus to detach from infected cells and to move through mucus and other bodily fluids. This enzyme is a major target of antiviral drugs used to treat influenza, such as oseltamivir (Tamiflu) and zanamivir (Relenza). Inhibiting the activity of neuraminidase can help to prevent the spread of the virus within the body and reduce the severity of symptoms.

Sialyltransferases are a group of enzymes that play a crucial role in the biosynthesis of sialic acids, which are a type of sugar molecule found on the surface of many cell types. These enzymes catalyze the transfer of sialic acid from a donor molecule (usually CMP-sialic acid) to an acceptor molecule, such as a glycoprotein or glycolipid.

The addition of sialic acids to these molecules can affect their function and properties, including their recognition by other cells and their susceptibility to degradation. Sialyltransferases are involved in various biological processes, including cell-cell recognition, inflammation, and cancer metastasis.

There are several different types of sialyltransferases, each with specific substrate preferences and functions. For example, some sialyltransferases add sialic acids to the ends of N-linked glycans, while others add them to O-linked glycans or glycolipids.

Abnormalities in sialyltransferase activity have been implicated in various diseases, including cancer, inflammatory disorders, and neurological conditions. Therefore, understanding the function and regulation of these enzymes is an important area of research with potential implications for disease diagnosis and treatment.

N-Acetylneuraminic Acid (Neu5Ac) is an organic compound that belongs to the family of sialic acids. It is a common terminal sugar found on many glycoproteins and glycolipids on the surface of animal cells. Neu5Ac plays crucial roles in various biological processes, including cell recognition, signaling, and intercellular interactions. It is also involved in the protection against pathogens by serving as a barrier to prevent their attachment to host cells. Additionally, Neu5Ac has been implicated in several disease conditions, such as cancer and inflammation, due to its altered expression and metabolism.

N-Acetylgalactosaminyltransferases (GalNAc-Ts) are a family of enzymes that play a crucial role in the process of protein glycosylation. Protein glycosylation is the attachment of carbohydrate groups, also known as glycans, to proteins. This modification significantly influences various biological processes such as protein folding, stability, trafficking, and recognition.

GalNAc-Ts specifically catalyze the transfer of N-acetylgalactosamine (GalNAc) from a donor molecule, UDP-GalNAc, to serine or threonine residues on acceptor proteins. This initial step of adding GalNAc to proteins is called mucin-type O-glycosylation and sets the stage for further glycan additions by other enzymes.

There are at least 20 different isoforms of GalNAc-Ts identified in humans, each with distinct substrate specificities, tissue distributions, and subcellular localizations. Aberrant expression or dysfunction of these enzymes has been implicated in various diseases, including cancer, where altered glycosylation patterns contribute to tumor progression and metastasis.

Glycolipids are a type of lipid (fat) molecule that contain one or more sugar molecules attached to them. They are important components of cell membranes, where they play a role in cell recognition and signaling. Glycolipids are also found on the surface of some viruses and bacteria, where they can be recognized by the immune system as foreign invaders.

There are several different types of glycolipids, including cerebrosides, gangliosides, and globosides. These molecules differ in the number and type of sugar molecules they contain, as well as the structure of their lipid tails. Glycolipids are synthesized in the endoplasmic reticulum and Golgi apparatus of cells, and they are transported to the cell membrane through vesicles.

Abnormalities in glycolipid metabolism or structure have been implicated in a number of diseases, including certain types of cancer, neurological disorders, and autoimmune diseases. For example, mutations in genes involved in the synthesis of glycolipids can lead to conditions such as Tay-Sachs disease and Gaucher's disease, which are characterized by the accumulation of abnormal glycolipids in cells.

Sulfoglycosphingolipids are a type of glycosphingolipid that contain a sulfate ester group in their carbohydrate moiety. They are important components of animal cell membranes and play a role in various biological processes, including cell recognition, signal transduction, and cell adhesion.

The most well-known sulfoglycosphingolipids are the sulfatides, which contain a 3'-sulfate ester on the galactose residue of the glycosphingolipid GalCer (galactosylceramide). Sulfatides are abundant in the nervous system and have been implicated in various neurological disorders.

Other sulfoglycosphingolipids include the seminolipids, which contain a 3'-sulfate ester on the galactose residue of lactosylceramide (Galβ1-4Glcβ1-Cer), and are found in high concentrations in the testis.

Abnormalities in sulfoglycosphingolipid metabolism have been associated with several genetic disorders, such as metachromatic leukodystrophy (MLD) and globoid cell leukodystrophy (GLD), which are characterized by progressive neurological deterioration.

Fast Atom Bombardment (FAB) Mass Spectrometry is a technique used for determining the mass of ions in a sample. In FAB-MS, the sample is mixed with a matrix material and then bombarded with a beam of fast atoms, usually xenon or cesium. This bombardment leads to the formation of ions from the sample which can then be detected and measured using a mass analyzer. The resulting mass spectrum provides information about the molecular weight and structure of the sample molecules. FAB-MS is particularly useful for the analysis of large, thermally labile, or polar molecules that may not ionize well by other methods.

Brain chemistry refers to the chemical processes that occur within the brain, particularly those involving neurotransmitters, neuromodulators, and neuropeptides. These chemicals are responsible for transmitting signals between neurons (nerve cells) in the brain, allowing for various cognitive, emotional, and physical functions.

Neurotransmitters are chemical messengers that transmit signals across the synapse (the tiny gap between two neurons). Examples of neurotransmitters include dopamine, serotonin, norepinephrine, GABA (gamma-aminobutyric acid), and glutamate. Each neurotransmitter has a specific role in brain function, such as regulating mood, motivation, attention, memory, and movement.

Neuromodulators are chemicals that modify the effects of neurotransmitters on neurons. They can enhance or inhibit the transmission of signals between neurons, thereby modulating brain activity. Examples of neuromodulators include acetylcholine, histamine, and substance P.

Neuropeptides are small protein-like molecules that act as neurotransmitters or neuromodulators. They play a role in various physiological functions, such as pain perception, stress response, and reward processing. Examples of neuropeptides include endorphins, enkephalins, and oxytocin.

Abnormalities in brain chemistry can lead to various neurological and psychiatric conditions, such as depression, anxiety disorders, schizophrenia, Parkinson's disease, and Alzheimer's disease. Understanding brain chemistry is crucial for developing effective treatments for these conditions.

Cerebrosides are a type of sphingolipid, which are lipids that contain sphingosine. They are major components of the outer layer of cell membranes and are particularly abundant in the nervous system. Cerebrosides are composed of a ceramide molecule (a fatty acid attached to sphingosine) and a sugar molecule, usually either glucose or galactose.

Glycosphingolipids that contain a ceramide with a single sugar residue are called cerebrosides. Those that contain more complex oligosaccharide chains are called gangliosides. Cerebrosides play important roles in cell recognition, signal transduction, and cell adhesion.

Abnormalities in the metabolism of cerebrosides can lead to various genetic disorders, such as Gaucher's disease, Krabbe disease, and Fabry disease. These conditions are characterized by the accumulation of cerebrosides or their breakdown products in various tissues, leading to progressive damage and dysfunction.

Carbohydrate conformation refers to the three-dimensional shape and structure of a carbohydrate molecule. Carbohydrates, also known as sugars, can exist in various conformational states, which are determined by the rotation of their component bonds and the spatial arrangement of their functional groups.

The conformation of a carbohydrate molecule can have significant implications for its biological activity and recognition by other molecules, such as enzymes or antibodies. Factors that can influence carbohydrate conformation include the presence of intramolecular hydrogen bonds, steric effects, and intermolecular interactions with solvent molecules or other solutes.

In some cases, the conformation of a carbohydrate may be stabilized by the formation of cyclic structures, in which the hydroxyl group at one end of the molecule forms a covalent bond with the carbonyl carbon at the other end, creating a ring structure. The most common cyclic carbohydrates are monosaccharides, such as glucose and fructose, which can exist in various conformational isomers known as anomers.

Understanding the conformation of carbohydrate molecules is important for elucidating their biological functions and developing strategies for targeting them with drugs or other therapeutic agents.

Guillain-Barré syndrome (GBS) is a rare autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nervous system, leading to muscle weakness, tingling sensations, and sometimes paralysis. The peripheral nervous system includes the nerves that control our movements and transmit signals from our skin, muscles, and joints to our brain.

The onset of GBS usually occurs after a viral or bacterial infection, such as respiratory or gastrointestinal infections, or following surgery, vaccinations, or other immune system triggers. The exact cause of the immune response that leads to GBS is not fully understood.

GBS typically progresses rapidly over days or weeks, with symptoms reaching their peak within 2-4 weeks after onset. Most people with GBS experience muscle weakness that starts in the lower limbs and spreads upward to the upper body, arms, and face. In severe cases, the diaphragm and chest muscles may become weakened, leading to difficulty breathing and requiring mechanical ventilation.

The diagnosis of GBS is based on clinical symptoms, nerve conduction studies, and sometimes cerebrospinal fluid analysis. Treatment typically involves supportive care, such as pain management, physical therapy, and respiratory support if necessary. In addition, plasma exchange (plasmapheresis) or intravenous immunoglobulin (IVIG) may be used to reduce the severity of symptoms and speed up recovery.

While most people with GBS recover completely or with minimal residual symptoms, some may experience long-term disability or require ongoing medical care. The prognosis for GBS varies depending on the severity of the illness and the individual's age and overall health.

Globosides are a type of glycosphingolipids, which are molecules that consist of a lipid and a carbohydrate. They are found in animal tissues, especially in the nervous system. The term "globoside" refers to a specific structure of these molecules, where the carbohydrate portion consists of a complex chain of sugars, including galactose, N-acetylgalactosamine, and glucose. Globosides play important roles in cell recognition and interaction, and abnormalities in their metabolism have been associated with certain diseases, such as paroxysmal nocturnal hemoglobinuria (PNH).

Ceramides are a type of lipid molecule that are found naturally in the outer layer of the skin (the stratum corneum). They play a crucial role in maintaining the barrier function and hydration of the skin. Ceramides help to seal in moisture, support the structure of the skin, and protect against environmental stressors such as pollution and bacteria.

In addition to their role in the skin, ceramides have also been studied for their potential therapeutic benefits in various medical conditions. For example, abnormal levels of ceramides have been implicated in several diseases, including diabetes, cardiovascular disease, and cancer. As a result, ceramide-based therapies are being investigated as potential treatments for these conditions.

Medically, ceramides may be mentioned in the context of skin disorders or diseases where there is a disruption in the skin's barrier function, such as eczema, psoriasis, and ichthyosis. In these cases, ceramide-based therapies may be used to help restore the skin's natural barrier and improve its overall health and appearance.

Gangliosidoses are a group of inherited metabolic disorders caused by the accumulation of certain complex lipids called gangliosides in the brain and nervous system. This buildup is due to a deficiency of specific enzymes needed to break down these substances. The three main types of gangliosidoses are:

1. Type 1 - Infantile Neurovisceral or Tay-Sachs Disease: Characterized by the absence of the enzyme hexosaminidase A, leading to severe neurological symptoms such as muscle weakness, blindness, and developmental delay in early infancy, with rapid progression and death usually occurring before age 4.
2. Type 2 - Juvenile or Subacute GM1 Gangliosidosis: Caused by a deficiency of the enzyme beta-galactosidase, resulting in progressive neurological symptoms such as motor and cognitive decline, beginning between ages 6 months and 2 years. Affected individuals may survive into adolescence or early adulthood.
3. Type 3 - Adult or Chronic GM1 Gangliosidosis: Characterized by a deficiency of beta-galactosidase, leading to milder neurological symptoms that appear in late childhood, adolescence, or even adulthood. The progression is slower compared to the other types, and life expectancy varies widely.

Gangliosidoses are autosomal recessive disorders, meaning an individual must inherit two copies of the defective gene (one from each parent) to develop the condition.

Tetanus toxin, also known as tetanospasmin, is a potent neurotoxin produced by the bacterium Clostridium tetani. This toxin binds to nerve endings and is transported to the nervous system's inhibitory neurons, where it blocks the release of inhibitory neurotransmitters, particularly glycine and GABA (gamma-aminobutyric acid). As a result, it causes uncontrolled muscle contractions or spasms, which are the hallmark symptoms of tetanus disease.

The toxin has two main components: an N-terminal portion called the light chain, which is the enzymatically active part that inhibits neurotransmitter release, and a C-terminal portion called the heavy chain, which facilitates the toxin's entry into neurons. The heavy chain also contains a binding domain that allows the toxin to recognize specific receptors on nerve cells.

Tetanus toxin is one of the most potent toxins known, with an estimated human lethal dose of just 2.5-3 nanograms per kilogram of body weight when introduced into the bloodstream. Fortunately, tetanus can be prevented through vaccination with the tetanus toxoid, which is part of the standard diphtheria-tetanus-pertussis (DTaP or Tdap) immunization series for children and adolescents and the tetanus-diphtheria (Td) booster for adults.

GM2 gangliosidoses are a group of inherited metabolic disorders caused by the accumulation of harmful amounts of GM2 gangliosides in the body's cells, particularly in the nerve cells of the brain. There are three main types of GM2 gangliosidoses: Tay-Sachs disease, Sandhoff disease, and AB variant of GM2 gangliosidosis. These conditions are characterized by progressive neurological degeneration, which can lead to severe physical and mental disabilities, and ultimately death in childhood or early adulthood.

The underlying cause of GM2 gangliosides is a deficiency in the enzyme hexosaminidase A (Tay-Sachs and AB variant) or both hexosaminidase A and B (Sandhoff disease), which are responsible for breaking down GM2 gangliosides. Without sufficient enzyme activity, GM2 gangliosides accumulate in the lysosomes of cells, leading to cell dysfunction and death.

Symptoms of GM2 gangliosidoses can vary depending on the specific type and severity of the disorder, but often include developmental delay, muscle weakness, loss of motor skills, seizures, blindness, and dementia. There is currently no cure for GM2 gangliosidoses, and treatment is focused on managing symptoms and improving quality of life.

Lipidoses are a group of genetic disorders characterized by abnormal accumulation of lipids (fats or fat-like substances) in various tissues and cells of the body due to defects in lipid metabolism. These disorders include conditions such as Gaucher's disease, Tay-Sachs disease, Niemann-Pick disease, Fabry disease, and Wolman disease, among others. The accumulation of lipids can lead to progressive damage in multiple organs, resulting in a range of symptoms and health complications. Early diagnosis and management are essential for improving the quality of life and prognosis of affected individuals.

Polyradiculoneuropathy is a medical term that refers to a condition affecting multiple nerve roots and peripheral nerves. It's a type of neuropathy, which is damage or disease affecting the peripheral nerves, and it involves damage to the nerve roots as they exit the spinal cord.

The term "poly" means many, "radiculo" refers to the nerve root, and "neuropathy" indicates a disorder of the nerves. Therefore, polyradiculoneuropathy implies that multiple nerve roots and peripheral nerves are affected.

This condition can result from various causes, such as infections (like Guillain-Barre syndrome), autoimmune disorders (such as lupus or rheumatoid arthritis), diabetes, cancer, or exposure to toxins. Symptoms may include weakness, numbness, tingling, or pain in the limbs, which can progress and become severe over time. Proper diagnosis and management are crucial for improving outcomes and preventing further nerve damage.

Cholera toxin is a protein toxin produced by the bacterium Vibrio cholerae, which causes the infectious disease cholera. The toxin is composed of two subunits, A and B, and its primary mechanism of action is to alter the normal function of cells in the small intestine.

The B subunit of the toxin binds to ganglioside receptors on the surface of intestinal epithelial cells, allowing the A subunit to enter the cell. Once inside, the A subunit activates a signaling pathway that results in the excessive secretion of chloride ions and water into the intestinal lumen, leading to profuse, watery diarrhea, dehydration, and other symptoms associated with cholera.

Cholera toxin is also used as a research tool in molecular biology and immunology due to its ability to modulate cell signaling pathways. It has been used to study the mechanisms of signal transduction, protein trafficking, and immune responses.

Carbohydrates are a major nutrient class consisting of organic compounds that primarily contain carbon, hydrogen, and oxygen atoms. They are classified as saccharides, which include monosaccharides (simple sugars), disaccharides (double sugars), oligosaccharides (short-chain sugars), and polysaccharides (complex carbohydrates).

Monosaccharides, such as glucose, fructose, and galactose, are the simplest form of carbohydrates. They consist of a single sugar molecule that cannot be broken down further by hydrolysis. Disaccharides, like sucrose (table sugar), lactose (milk sugar), and maltose (malt sugar), are formed from two monosaccharide units joined together.

Oligosaccharides contain a small number of monosaccharide units, typically less than 20, while polysaccharides consist of long chains of hundreds to thousands of monosaccharide units. Polysaccharides can be further classified into starch (found in plants), glycogen (found in animals), and non-starchy polysaccharides like cellulose, chitin, and pectin.

Carbohydrates play a crucial role in providing energy to the body, with glucose being the primary source of energy for most cells. They also serve as structural components in plants (cellulose) and animals (chitin), participate in various metabolic processes, and contribute to the taste, texture, and preservation of foods.

"Cattle" is a term used in the agricultural and veterinary fields to refer to domesticated animals of the genus *Bos*, primarily *Bos taurus* (European cattle) and *Bos indicus* (Zebu). These animals are often raised for meat, milk, leather, and labor. They are also known as bovines or cows (for females), bulls (intact males), and steers/bullocks (castrated males). However, in a strict medical definition, "cattle" does not apply to humans or other animals.

Miller Fisher Syndrome (MFS) is a rare neurological disorder that is considered a variant of Guillain-Barré syndrome. It is characterized by the triad of symptoms including ophthalmoplegia (paralysis of the eye muscles), ataxia (loss of coordination and balance), and areflexia (absence of reflexes). Some patients may also experience weakness or paralysis in the limbs, and some cases may involve bulbar symptoms such as dysphagia (difficulty swallowing) and dysarthria (slurred speech). The syndrome is caused by an immune response that damages the nerves, and it often follows a viral infection. Treatment typically includes supportive care, plasma exchange, or intravenous immunoglobulin therapy to help reduce the severity of the symptoms.

Glucosylceramides are a type of glycosphingolipid, which are complex lipids found in the outer layer of cell membranes. They consist of a ceramide molecule (a fatty acid and sphingosine) with a glucose molecule attached to it through a glycosidic bond.

Glucosylceramides play important roles in various cellular processes, including cell signaling, membrane structure, and cell-to-cell recognition. They are particularly abundant in the nervous system, where they contribute to the formation of the myelin sheath that surrounds nerve fibers.

Abnormal accumulation of glucosylceramides is associated with certain genetic disorders, such as Gaucher disease and Krabbe disease, which are characterized by neurological symptoms and other health problems. Enzyme replacement therapy or stem cell transplantation may be used to treat these conditions.

Molecular sequence data refers to the specific arrangement of molecules, most commonly nucleotides in DNA or RNA, or amino acids in proteins, that make up a biological macromolecule. This data is generated through laboratory techniques such as sequencing, and provides information about the exact order of the constituent molecules. This data is crucial in various fields of biology, including genetics, evolution, and molecular biology, allowing for comparisons between different organisms, identification of genetic variations, and studies of gene function and regulation.

Sphingosine is not a medical term per se, but rather a biological compound with importance in the field of medicine. It is a type of sphingolipid, a class of lipids that are crucial components of cell membranes. Sphingosine itself is a secondary alcohol with an amino group and two long-chain hydrocarbons.

Medically, sphingosine is significant due to its role as a precursor in the synthesis of other sphingolipids, such as ceramides, sphingomyelins, and gangliosides, which are involved in various cellular processes like signal transduction, cell growth, differentiation, and apoptosis (programmed cell death).

Moreover, sphingosine-1-phosphate (S1P), a derivative of sphingosine, is an important bioactive lipid mediator that regulates various physiological functions, including immune response, vascular maturation, and neuronal development. Dysregulation of S1P signaling has been implicated in several diseases, such as cancer, inflammation, and cardiovascular disorders.

In summary, sphingosine is a crucial biological compound with medical relevance due to its role as a precursor for various sphingolipids involved in cellular processes and as a precursor for the bioactive lipid mediator S1P.

Glycoconjugates are a type of complex molecule that form when a carbohydrate (sugar) becomes chemically linked to a protein or lipid (fat) molecule. This linkage, known as a glycosidic bond, results in the formation of a new molecule that combines the properties and functions of both the carbohydrate and the protein or lipid component.

Glycoconjugates can be classified into several categories based on the type of linkage and the nature of the components involved. For example, glycoproteins are glycoconjugates that consist of a protein backbone with one or more carbohydrate chains attached to it. Similarly, glycolipids are molecules that contain a lipid anchor linked to one or more carbohydrate residues.

Glycoconjugates play important roles in various biological processes, including cell recognition, signaling, and communication. They are also involved in the immune response, inflammation, and the development of certain diseases such as cancer and infectious disorders. As a result, understanding the structure and function of glycoconjugates is an active area of research in biochemistry, cell biology, and medical science.

The brain is the central organ of the nervous system, responsible for receiving and processing sensory information, regulating vital functions, and controlling behavior, movement, and cognition. It is divided into several distinct regions, each with specific functions:

1. Cerebrum: The largest part of the brain, responsible for higher cognitive functions such as thinking, learning, memory, language, and perception. It is divided into two hemispheres, each controlling the opposite side of the body.
2. Cerebellum: Located at the back of the brain, it is responsible for coordinating muscle movements, maintaining balance, and fine-tuning motor skills.
3. Brainstem: Connects the cerebrum and cerebellum to the spinal cord, controlling vital functions such as breathing, heart rate, and blood pressure. It also serves as a relay center for sensory information and motor commands between the brain and the rest of the body.
4. Diencephalon: A region that includes the thalamus (a major sensory relay station) and hypothalamus (regulates hormones, temperature, hunger, thirst, and sleep).
5. Limbic system: A group of structures involved in emotional processing, memory formation, and motivation, including the hippocampus, amygdala, and cingulate gyrus.

The brain is composed of billions of interconnected neurons that communicate through electrical and chemical signals. It is protected by the skull and surrounded by three layers of membranes called meninges, as well as cerebrospinal fluid that provides cushioning and nutrients.

A genome is the complete set of genetic material (DNA, or in some viruses, RNA) present in a single cell of an organism. It includes all of the genes, both coding and noncoding, as well as other regulatory elements that together determine the unique characteristics of that organism. The human genome, for example, contains approximately 3 billion base pairs and about 20,000-25,000 protein-coding genes.

The term "genome" was first coined by Hans Winkler in 1920, derived from the word "gene" and the suffix "-ome," which refers to a complete set of something. The study of genomes is known as genomics.

Understanding the genome can provide valuable insights into the genetic basis of diseases, evolution, and other biological processes. With advancements in sequencing technologies, it has become possible to determine the entire genomic sequence of many organisms, including humans, and use this information for various applications such as personalized medicine, gene therapy, and biotechnology.

Molecular sequence annotation is the process of identifying and describing the characteristics, functional elements, and relevant information of a DNA, RNA, or protein sequence at the molecular level. This process involves marking the location and function of various features such as genes, regulatory regions, coding and non-coding sequences, intron-exon boundaries, promoters, introns, untranslated regions (UTRs), binding sites for proteins or other molecules, and post-translational modifications in a given molecular sequence.

The annotation can be manual, where experts curate and analyze the data to predict features based on biological knowledge and experimental evidence. Alternatively, computational methods using various bioinformatics tools and algorithms can be employed for automated annotation. These tools often rely on comparative analysis, pattern recognition, and machine learning techniques to identify conserved sequence patterns, motifs, or domains that are associated with specific functions.

The annotated molecular sequences serve as valuable resources in genomic and proteomic studies, contributing to the understanding of gene function, evolutionary relationships, disease associations, and biotechnological applications.

A genetic database is a type of biomedical or health informatics database that stores and organizes genetic data, such as DNA sequences, gene maps, genotypes, haplotypes, and phenotype information. These databases can be used for various purposes, including research, clinical diagnosis, and personalized medicine.

There are different types of genetic databases, including:

1. Genomic databases: These databases store whole genome sequences, gene expression data, and other genomic information. Examples include the National Center for Biotechnology Information's (NCBI) GenBank, the European Nucleotide Archive (ENA), and the DNA Data Bank of Japan (DDBJ).
2. Gene databases: These databases contain information about specific genes, including their location, function, regulation, and evolution. Examples include the Online Mendelian Inheritance in Man (OMIM) database, the Universal Protein Resource (UniProt), and the Gene Ontology (GO) database.
3. Variant databases: These databases store information about genetic variants, such as single nucleotide polymorphisms (SNPs), insertions/deletions (INDELs), and copy number variations (CNVs). Examples include the Database of Single Nucleotide Polymorphisms (dbSNP), the Catalogue of Somatic Mutations in Cancer (COSMIC), and the International HapMap Project.
4. Clinical databases: These databases contain genetic and clinical information about patients, such as their genotype, phenotype, family history, and response to treatments. Examples include the ClinVar database, the Pharmacogenomics Knowledgebase (PharmGKB), and the Genetic Testing Registry (GTR).
5. Population databases: These databases store genetic information about different populations, including their ancestry, demographics, and genetic diversity. Examples include the 1000 Genomes Project, the Human Genome Diversity Project (HGDP), and the Allele Frequency Net Database (AFND).

Genetic databases can be publicly accessible or restricted to authorized users, depending on their purpose and content. They play a crucial role in advancing our understanding of genetics and genomics, as well as improving healthcare and personalized medicine.

DNA Sequence Analysis is the systematic determination of the order of nucleotides in a DNA molecule. It is a critical component of modern molecular biology, genetics, and genetic engineering. The process involves determining the exact order of the four nucleotide bases - adenine (A), guanine (G), cytosine (C), and thymine (T) - in a DNA molecule or fragment. This information is used in various applications such as identifying gene mutations, studying evolutionary relationships, developing molecular markers for breeding, and diagnosing genetic diseases.

The process of DNA Sequence Analysis typically involves several steps, including DNA extraction, PCR amplification (if necessary), purification, sequencing reaction, and electrophoresis. The resulting data is then analyzed using specialized software to determine the exact sequence of nucleotides.

In recent years, high-throughput DNA sequencing technologies have revolutionized the field of genomics, enabling the rapid and cost-effective sequencing of entire genomes. This has led to an explosion of genomic data and new insights into the genetic basis of many diseases and traits.

Genomics is the scientific study of genes and their functions. It involves the sequencing and analysis of an organism's genome, which is its complete set of DNA, including all of its genes. Genomics also includes the study of how genes interact with each other and with the environment. This field of study can provide important insights into the genetic basis of diseases and can lead to the development of new diagnostic tools and treatments.

Gangliosides at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Overview of gangliosides at lipidlibrary. ... Natural and semisynthetic gangliosides are considered possible therapeutics for neurodegenerative disorders. Gangliosides are ... More than 60 gangliosides are known, which differ from each other mainly in the position and number of NANA residues. It is a ... A ganglioside is a molecule composed of a glycosphingolipid (ceramide and oligosaccharide) with one or more sialic acids (e.g. ...
In organic chemistry, GM2 is a type of ganglioside. G refers to ganglioside, the M is for monosialic (as in it has one sialic ... Ganglioside GM2 activator protein Sphingolipidoses Structures of GM1, GM2, GM3 gangliosides Guetta E, Peleg L (2008). Rapid ... Ganglioside+GM2 at the U.S. National Library of Medicine Medical Subject Headings (MeSH) (Articles without KEGG source, ... acid), and 2 refers to the fact that it was the second monosialic ganglioside discovered. It is associated with GM2 ...
In enzymology, a ganglioside galactosyltransferase (EC 2.4.1.62) is an enzyme that catalyzes the chemical reaction UDP- ... Yip GB, Dain JA (1970). "The enzymic synthesis of ganglioside. II. UDP-galactose: N-acetylgalactosaminyl-(N-acetylneuraminyl) ... UDP-galactose-GM2 ganglioside galactosyltransferase, and GM1-synthase. This enzyme participates in glycosphingolipid ... "Conversion of Tay-Sachs ganglioside to monosialoganglioside by brain uridine diphosphate D-galactose: glycolipid ...
... they are not always available for each ganglioside. Therefore, indirect measurement of ganglioside expression by quantifying ... GD1a, ganglioside also serves as SeV receptor and is found in large quantities on the surfaces of breast cancer stem cells. ... "Correction: Specific Gangliosides Function as Host Cell Receptors for Sendai Virus". Proceedings of the National Academy of ... For example, GD1a, which is a ganglioside and sialylated glycan (glycolipid), is found in large quantities on the surfaces of ...
Separation of membranes with different ratios of ganglioside sialylating activity to gangliosides". Biochem. J. 168 (3): 325-32 ... Landa CA, Maccioni HJ, Arce A, Caputto R (1977). "The biosynthesis of brain gangliosides. ...
Higuchi, Ryuichi; Inagaki, Masanori; Yamada, Koji; Miyamoto, Tomofumi (2007). "Biologically active gangliosides from ...
Seyfried, Thomas N.; Yu, Robert K.; Miyazawa, Nobuko; Lai, Yin‐Lok (1982). "Retinal Gangliosides in RCS Mutant Rats". Journal ...
Gangliosides: the most complex animal glycolipids. They contain negatively charged oligosacchrides with one or more sialic acid ... Ariga T, McDonald MP, Yu RK (June 2008). "Role of ganglioside metabolism in the pathogenesis of Alzheimer's disease--a review ... They may be as complicated a set of compounds as the negatively charged gangliosides in animals. Glycophosphatidylinositols: a ... residues; more than 200 different gangliosides have been identified. They are most abundant in nerve cells. Globosides: ...
Certain aspects of lipid rafts inhibit EGF receptor function: the ganglioside component of lipid rafts was shown to inhibit ... For example, fluorophores conjugated to cholera-toxin B-subunit, which binds to the raft constituent ganglioside GM1 is used ... Miljan, Erik A; Bremer, Eric G. (2002). "Regulation of growth factor receptors by gangliosides". Sci STKE. 2002 (160): RE15. ... ganglioside GM1 located in lipid rafts and major histocompatibility (MHC) class I molecule. Binding of SV40 with MHC class I ...
The molecule GM2 ganglioside occurs normally but, due to an enzyme defect, builds up pathologically in the cells of victims of ... At the beginning of the 1960s, Klenk again made gangliosides the focus of his work. He elucidated the structures of many ... Klenk, Ernst (1942). "Über die Ganglioside des Gehirns bei der infantilen amaurotischen Idiotie vom Typ Tay-Sachs". Berichte ... In 1935 Klenk discovered a new group of glycosphingolipids in the nervous tissue, which he called gangliosides. ( ...
GM2A (GM2 ganglioside activator) has been viewed as a member of the SAP family and has been called SAP-3 (sphingolipid ... Hiraiwa M, Soeda S, Kishimoto Y, O'Brien JS (1993). "Binding and transport of gangliosides by prosaposin". Proc. Natl. Acad. ... 1990). "The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein ... GM1 ganglioside activator, dispersin, and nonspecific. It has been observed that this particular saposin activates many enzymes ...
GM1 gangliosides are found in lipid rafts on the cell surface. B subunit complexes labelled with fluorescent tags or ... Enterotoxin Ganglioside Ryan KJ; Ray CG, eds. (2004). Sherris Medical Microbiology (4th ed.). McGraw Hill. p. 375. ISBN 978-0- ... Cholera toxin acts by the following mechanism: First, the B subunit ring of the cholera toxin binds to GM1 gangliosides on the ... 2018). "GM1 ganglioside-independent intoxication by Cholera toxin". PLOS Pathogens. 14 (2): e1006862. doi:10.1371/journal.ppat. ...
Holmgren, J.; Lönnroth, I.; Månsson, J.; Svennerholm, L. (1975). "Interaction of cholera toxin and membrane GM! ganglioside of ... the GM1 ganglioside, then the first-ever structurally defined biologic receptor molecule. They were also the first to describe ...
... s include: Cerebrosides Gangliosides Globosides Gangliosides are mainly found in the cell membranes of the ... However, gangliosides are also found in other cells. Sialic acids are, for example, part of the so-called sialyl-Lewis-x ... While in gangliosides sialic acids are found, sulfatides have a sulfate group. The structural similarity of most glycolipids is ... in case of gangliosides it affects the gangliosidoses (e.g. Tay-Sachs disease) . Sphingomyelin Charles Chalfant; Maurizio Del ...
Gangliosides are membrane-bound glycosphingolipids containing sialic acid. Ganglioside GD3 is known to be important for cell ... 2006). "Fundamental study of small interfering RNAs for ganglioside GD3 synthase gene as a therapeutic target of lung cancers ... 1997). "Requirement for GD3 ganglioside in CD95- and ceramide-induced apoptosis". Science. 277 (5332): 1652-5. doi:10.1126/ ... is a type II membrane protein that catalyzes the transfer of sialic acid from CMP-sialic acid to GM3 to produce gangliosides ...
... uridine diphosphoacetylgalactosamine-ganglioside GM3 acetylgalactosaminyltransferase, ganglioside GM2 synthase, ganglioside GM3 ... Dicesare JL, Dain JA (March 1971). "The enzymic synthesis of ganglioside. IV. UDP-N-acetylgalactosamine: (N-acetylneuraminyl)- ... "Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions". ... ceramide This enzyme catalyses the formation of the gangliosides (i.e. sialic-acid-containing glycosphingolipids) GM2, GD2 and ...
Anti-ganglioside antibodies "Mitumomab - AdisInsight". Adisinsight.springer.com. 2005-06-13. Retrieved 2017-01-06. Clinical ...
Her thesis is titled "Structure and Function of Gangliosides". Between 1995 and 1997 she was head of the Department of Clinical ...
MPyV capsid protein VP1 binds to sialic acids of gangliosides GD1a and GT1b on the cell surface. The functions of VP2 and VP3 ... Tsai, B; Gilbert, JM; Stehle, T; Lencer, W; Benjamin, TL; Rapoport, TA (1 September 2003). "Gangliosides are receptors for ...
GM2 and GD2 gangliosides are sialic acid-containing glycosphingolipids. GalNAc-T is the enzyme involved in the biosynthesis of ... 4 N-acetylgalactosaminyltransferase cDNAs that determine the expression of GM2 and GD2 gangliosides". J Biol Chem. 267 (17): ... 4 N-acetylgalactosaminyltransferase cDNAs that determine the expression of GM2 and GD2 gangliosides". J. Biol. Chem. 269 (9): ...
Both the ganglioside and the GPI-anchored protein are located in lipid microdomains and both are requisite for specific TeNT ... Winter, A; Ulrich, WP; Wetterich, F; Weller, U; Galla, HJ (17 June 1996). "Gangliosides in phospholipid bilayer membranes: ...
Tsai B, Gilbert JM, Stehle T, Lencer W, Benjamin TL, Rapoport TA (September 2003). "Gangliosides are receptors for murine ... including some gangliosides, on the surfaces of cells to initiate the process of viral infection. The VP1 protein, along with ... including some gangliosides, on the cell surface. Canonically, VP1 interacts specifically with α(2,3)-linked and α(2,6)-linked ...
... (monosialotetrahexosylganglioside) the "prototype" ganglioside, is a member of the ganglio series of gangliosides which ... Sphingolipidoses Structures of GM1, GM2, GM3 gangliosides Mocchetti I (2005). "Exogenous gangliosides, neuronal plasticity and ... The secreted toxin attaches to the surface of the host mucosa cell by binding to GM1 gangliosides. GM1 consists of a sialic ... 1994). "IgM ganglioside GM1 antibodies in patients with autoimmune disease or neuropathy, and controls". J. Clin. Pathol. 47 (4 ...
Antibodies to ganglioside are found to be elevated in coeliac disease. Recent studies show that gliadin can cross-link to ... IgA to gangliosides have been observed in Guillain-Barré syndrome. IgM. IgM antibodies have been detected in early work, but ... Autoantigenic gangliosides that are currently known are GD3, GM1, GQ3 and GT1. Anti-GD3 antibodies have been found in ... 1994). "IgM ganglioside GM1 antibodies in patients with autoimmune disease or neuropathy, and controls". J. Clin. Pathol. 47 (4 ...
Horowitz SH (1984). "Ganglioside (Cronassial) Therapy in Diabetic Neuropathy". Ganglioside Structure, Function, and Biomedical ... Beginning in 1975, she supported the drug Cronassial (a particular mixture of gangliosides) produced by Fidia from bovine brain ... Staughton RC, Good J (1990). "Double-blind, placebo-controlled clinical trial of a mixture of gangliosides ('Cronassial') in ... While working for Fidia, she improved the understanding of gangliosides. ...
The mutation disrupts the activity of the enzyme, which results in the build-up of the molecule GM2 ganglioside within cells, ... The hydrolysis of GM2-ganglioside requires three proteins. Two of them are subunits of hexosaminidase A; the third is a small ... This red spot is a retinal area that appears red because of gangliosides in the surrounding retinal ganglion cells. The ... Deficiency in any one of these proteins leads to ganglioside storage, primarily in the lysosomes of neurons. Tay-Sachs disease ...
... (monosialodihexosylganglioside) is a type of ganglioside. The letter G refers to ganglioside, and M is for monosialic acid ... GM3 serves as a precursor for other, more complex gangliosides. Like other gangliosides, GM3 is synthesized in the Golgi ... Chung TW, Choi HJ, Kim SJ, Kwak CH, Song KH, Jin UH, Chang YC, Chang HW, Lee YC, Ha KT, Kim CH (2014-05-14). "The ganglioside ... Wang H, Isaji T, Satoh M, Li D, Arai Y, Gu J (January 2013). "Antitumor effects of exogenous ganglioside GM3 on bladder cancer ...
Bachhawat has contributed to the knowledge on the metabolism of mucopolysaccharides, gangliosides and cerebrosulphatides, ... "Interaction between lectin from Ricinus communis and liposome containing gangliosides". Nature. 257 (5529): 802-804. Bibcode: ...
There he isolated two glysosphingolipids and studied antibodies to gangliosides. These findings were useful to further ... Rapport, Maurice (1990). "Implications of Altered Brain Ganglioside Profiles in Amyotrophic Lateral Sclerosis (ALS)" (PDF). ...
Shirure VS, Henson KA, Schnaar RL, Nimrichter L, Burdick MM (March 2011). "Gangliosides expressed on breast cancer cells are E- ... and gangliosides were identified as E-selectin ligands present on breast cancer cells. On human neutrophils the ...
Gangliosides at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Overview of gangliosides at lipidlibrary. ... Natural and semisynthetic gangliosides are considered possible therapeutics for neurodegenerative disorders. Gangliosides are ... More than 60 gangliosides are known, which differ from each other mainly in the position and number of NANA residues. It is a ... A ganglioside is a molecule composed of a glycosphingolipid (ceramide and oligosaccharide) with one or more sialic acids (e.g. ...
GDAP2 ganglioside induced differentiation associated protein 2 [Homo sapiens] GDAP2 ganglioside induced differentiation ... ganglioside induced differentiation associated protein 2provided by HGNC. Primary source. HGNC:HGNC:18010 See related. Ensembl: ... GDAP2 ganglioside induced differentiation associated protein 2 [ Homo sapiens (human) ] Gene ID: 54834, updated on 7-Sep-2023 ... NM_001135589.3 → NP_001129061.1 ganglioside-induced differentiation-associated protein 2 isoform b ...
Gangliosides. Gangliosides are acidic glycosphingolipids that contribute a substantial presence to the outer leaflet of the ... 3 A deficiency in the enzymes responsible for metabolizing gangliosides causes toxic levels of ganglioside accumulation which ... S. Birkle, G. Zeng, L. Gao, R.K. Yu, and J. Aubry "Role of tumor-associated gangliosides in cancer progression" Biochimie, Vol ... J. Gu, C. Tifft and S. Soldin "Simultaneous quantification of GM1 and GM2 gangliosides by isotope dilution tandem mass ...
Antibodies for proteins involved in ganglioside biosynthetic process pathways, according to their Panther/Gene Ontology ... Antibodies for proteins involved in ganglioside biosynthetic process pathways; according to their Panther/Gene Ontology ...
Gangliosides, Glycosphingolipids, HILIC, In-source dissociation, Mass spectrometry Abstract. Gangliosides are a family of ... Challenges in the Analysis of Gangliosides by LC-MS Authors. * Thomas Muggli University of Bern, Department of Chemistry, ... Gangliosides are of high abundance in neuronal tissues and are involved in numerous biological processes, such as cell-cell ... Alteration of the ganglioside profile is associated with various neurodegenerative diseases and there is indication that ...
Nevertheless, without an anti-ganglioside antibody assay, in Korea AMAN is frequently misdiagnosed as AIDP by single ... Anti-ganglioside antibodies were frequently found in the serum of Korean GBS patients, and each antibody was correlated ... Conclusions: Anti-ganglioside antibodies were frequently found in the serum of Korean GBS patients, and each antibody was ... Prevalence of anti-ganglioside antibodies and their clinical correlates with guillain-barré syndrome in Korea: a nationwide ...
DRGs are double-labeled for GD1a ganglioside (green) in A-F, H-M (or GT1b ganglioside in O-T) and CTB for GM1 ganglioside (red ... and GM1 gangliosides (CTB, red). The ratio of GM1/GD1a gangliosides (± SEM) was plotted in G, with the GM1 and GD1a ganglioside ... Sialidases, such as Neu3, act to remove terminal sialic acid groups, converting gangliosides, such as the main CNS gangliosides ... Because Neu3 sialidase activation converts more complex gangliosides to GM1 ganglioside and because GM1 has been shown to ...
Based on these findings, it has been suggested that C jejuni components mimic human gangliosides GM1 and GD1a, and C jejuni ... There is now good evidence that gangliosides or similar components trigger the development of axonal GBS.2 Axonal GBS ... Contamination with gangliosides in brain-derived rabies vaccine may trigger Guillain-Barré syndrome ... Contamination with gangliosides in brain-derived rabies vaccine may trigger Guillain-Barré syndrome ...
... it is possible that simpler gangliosides are able to compensate for the lack of complex gangliosides.. Axonal density and ... which lack b-series gangliosides but have increased levels of a-series gangliosides. These findings suggest that complex ... mice lacking all complex gangliosides, suggesting that complex gangliosides modulate the formation of nodes of Ranvier. In ... Gangliosides have also been shown to have neuroprotective actions and have been considered as candidates for the treatment of ...
Lack of antibody response to Guillain-Barré syndrome-related gangliosides in mice and men after novel flu vaccination ... Lack of antibody response to Guillain-Barré syndrome-related gangliosides in mice and men after novel flu vaccination ... Lack of antibody response to Guillain-Barré syndrome-related gangliosides in mice and men after novel flu vaccination ...
EP-0433113-B1 chemical patent summary.
EP-1517692-B1 chemical patent summary.
Poster presentation: Changes in Guillain-Barre Syndrome-Associated Ganglioside Antibody Test Results at a National Clinical ... Changes in Guillain-Barre Syndrome-Associated Ganglioside Antibody Test Results at a National Clinical Laboratory During the ... Changes in Guillain-Barre Syndrome-Associated Ganglioside Antibody Test Results at a National Clinical Laboratory During the ...
Ganglioside GD3 and its mimetics induce cytochrome c release from mitochondria: Y. Inoki, et al.; BBRC 276, 1210 (2000), ... Involvement of ganglioside GT1b in glutamate release from neuroblastoma cells: S. Watanabe, et al.; Neurosci. Lett. 517, 140 ( ... Ganglioside GD3, the mitochondrial permeability transition, and apoptosis: B.S. Kristal & A.M. Brown; Ann. NY Acad. Sci. 893, ... Apoptogenic ganglioside GD3 directly induces the mitochondrial permeability transition: B.S. Kristal & A.M. Brown; J. Biol. ...
LOINC Group Code LG12484-8 Ganglioside GM1 Ab.IgG+IgM,PrThr,Pt,ANYBldSerPl ... Ganglioside GM1 IgG+IgM Ab [Presence] in Serum Archetype. 63244-8. Ganglioside GM1 IgG+IgM Ab [Presence] in Serum by ... Deprecated Ganglioside GM1 IgG+IgM Ab [Presence] in Serum by Immunoblot. 89519-3. Ganglioside GM1 IgG+IgM Ab [Presence] in ... Ganglioside GM1 Ab.IgG+IgM,PrThr,Pt,ANYBldSerPl Active Maturity: Beta *The LOINC Groups project is a work in progress. The ...
Natural occurrence of ganglioside lactones. Isolation and characterization of GD1b inner ester from adult human brain: L. ... Anti-inflammatory role of GM1 and other gangliosides on microglia: D. Galleguillos, et al.; J. Neuroinflammation 19, 9 (2022), ... Ganglioside GD1b . disodium salt (bovine brain) - ALX-302-009 ...
Out of all the gangliosides, GM1 not only is the most abundant ganglioside in the human body, but the antibodies (both isotypes ... Given the structural similarities among different gangliosides on the microtiter plate, anti-GM1 was used as a surrogate ...
Ganglioside GM1[d18:1, (Carbon-13)C16:0]
... Houliston, R ... The display by DH1, of a surface glycan that mimics the terminal trisaccharide portion of disialosyl-containing gangliosides, ...
mouse Ganglioside GM1 antibody validated for ELISA,Immunofluorescence,Immunohistochemistry,Western Blot ... Localization of major gangliosides in the PNS: implications for immune neuropathies.. Sheikh KA Brain : a journal of neurology ... Localization of major gangliosides in the PNS: implications for immune neuropathies.. Sheikh KA Brain : a journal of neurology ... Localization of major gangliosides in the PNS: implications for immune neuropathies.. Sheikh KA Brain : a journal of neurology ...
This meta-analysis aimed to evaluate the efficacy and safety of Ganglioside-monosialic acid (GM1) in preventing CIPN. ... From: Ganglioside-monosialic acid (GM1) for prevention of chemotherapy-induced peripheral neuropathy: a meta-analysis with ...
... it is possible that they may express different gangliosides. We analyzed the ganglioside profiles of primary uveal melanoma in ... "Variations in the ganglioside profile of uveal melanoma correlate with cytologic heterogeneity." Int J Cancer, vol. 52, no. 5, ... "Variations in the ganglioside profile of uveal melanoma correlate with cytologic heterogeneity." Int J Cancer 52, no. 5 ( ... Variations in the ganglioside profile of uveal melanoma correlate with cytologic heterogeneity.. Publication , Journal Article ...
Gangliosides purification with liquid chromatography methods. Packing materials and technologies are supported by GALAK ... Gangliosides (GM1) Purification. Gangliosides are nerve growth, stable biofilm development and regeneration of the nervous ... Normally, gangliosides are extracted and purified from animals. This method use reverse phase chromatography to enrich and ... Bettsep® BP70 filler can effectively enrich and purify the active components in the ganglioside extract with a purity of more ...
title = "Expression of globe-series gangliosides in human renal cell carcinoma",. abstract = "Gangliosides have been shown to ... Saito S, Orikasa S, Satoh M, Ohyama C, Ito A, Takahashi T. Expression of globe-series gangliosides in human renal cell ... Saito, S, Orikasa, S, Satoh, M, Ohyama, C, Ito, A & Takahashi, T 1997, Expression of globe-series gangliosides in human renal ... Saito, S., Orikasa, S., Satoh, M., Ohyama, C., Ito, A., & Takahashi, T. (1997). Expression of globe-series gangliosides in ...
Test ID: GANGLIOSIDE ANTIBODIES PANEL IGG - NNI. Price: SGD 590.44 W/GST ...
... gangliosides, and carnitine with 113 fully reprogrammed pathways. The observed changes are in accordance with literature ... 2.4.4. Gangliosides are Involved in Adipocyte Differentiation and Insulin Sensitivity. The m/z for the gangliosides GM3(d18:1/ ... Figure A7. MS2 spectra of predicted ganglioside. Fragmentation spectra (Thermo Xcalibur) of regulated ganglioside GM3(d18:1/16: ... Figure A7. MS2 spectra of predicted ganglioside. Fragmentation spectra (Thermo Xcalibur) of regulated ganglioside GM3(d18:1/16: ...
Be the first to review "For immunochemical detection of ganglioside GD3 50 µL" Cancel reply. Your email address will not be ... For immunochemical detection of ganglioside GD3 50 µL ... For immunochemical detection of ganglioside GD3 50 µL quantity ...
The preferential binding of 7c11.e8 to melanoma tissues and the reactivity with two of the major melanoma gangliosides (GM3 and ... "Cell surface reactive human monoclonal antibody directed to human melanoma-associated gangliosides." Melanoma Res, vol. 3, no. ... Cell surface reactive human monoclonal antibody directed to human melanoma-associated gangliosides.. Publication , Journal ... "Cell surface reactive human monoclonal antibody directed to human melanoma-associated gangliosides." Melanoma Res 3, no. 6 ( ...
Objective To explore the feasibility of ganglioside GD2 as a marker f... ... Ganglioside GD2 is a potential candidate marker for lung cancer stem cells LI Zhen, DUAN Zhaojun*, LUO Yunping* ... Conclusions Ganglioside GD2 is a potential marker for LCSCs to identify cancer stem cells, providing a novel strategy of GD2- ... Abstract: Objective To explore the feasibility of ganglioside GD2 as a marker for lung cancer stem cells (LCSCs). Methods ...
  • Guillain-Barré syndrome, which has been linked to the production of anti-ganglioside antibodies. (wikipedia.org)
  • No previous studies have investigated the relationship between various anti-ganglioside antibodies and the clinical characteristics of Guillain-Barré syndrome (GBS) in Korea. (nih.gov)
  • The aim of this study was to determine the prevalence and types of anti-ganglioside antibodies in Korean GBS patients, and to identify their clinical significance. (nih.gov)
  • Anti-ganglioside antibodies were frequently found in the serum of Korean GBS patients, and each antibody was correlated strongly with the various clinical manifestations. (nih.gov)
  • 2 Axonal GBS associated with IgG anti-GM1 or anti-GD1a antibodies after bovine brain ganglioside administration have been recorded in several patients. (bmj.com)
  • Out of all the gangliosides, GM1 not only is the most abundant ganglioside in the human body, but the antibodies (both isotypes: IgG and IgM) directed against GM1 are the most frequently tested in large case series from daily routine. (buhlmannlabs.ch)
  • Immunoglobulin G-class mouse monoclonal antibodies to major brain gangliosides. (uiowa.edu)
  • We attempted to produce specific antibodies to various gangliosides by immunizing chickens. (elsevierpure.com)
  • Antibodies to gangliosides, no. (cdc.gov)
  • Here, we show that the peptides have overlapping binding sites on the mAb, 14G2a and restricted specificity, as they did not crossreact with other ganglioside-specific antibodies tested. (edu.pl)
  • Methods: Serum samples from 129 Agricultur al Health Study swine farm ers (some of whom also worked with other animals) and 46 non- farm ers, all from Iowa, were analyzed for anti-C. jejuni antibodies and anti-ganglioside autoantibodies using ELISA. (cdc.gov)
  • Most of these patients have antibodies against the GQ1b ganglioside. (medscape.com)
  • Cerebrospinal fluid anti-GM1 ganglioside antibodies and oligoclonal band were negative and the IgG index was normal. (who.int)
  • Gangliosides are acidic glycosphingolipids that contribute a substantial presence to the outer leaflet of the cell plasma membrane. (matreya.com)
  • Gangliosides, complex glycosphingolipids on the plasma membrane containing one or more sialic acid residues, are key components of microdomains. (jneurosci.org)
  • Gangliosides are a family of sialic acid-containing glycosphingolipids that are enriched in the nervous system. (gla.ac.uk)
  • Gangliosides are glycosphingolipids with one or more sialic acids. (uiowa.edu)
  • GM2 and GD2 gangliosides are sialic acid-containing glycosphingolipids. (nih.gov)
  • The adult mammalian nervous system has four dominant types of gangliosides: GM1, GD1a, GD1b, and GT1b. (jneurosci.org)
  • We demonstrated here that exposure of neuronal cells to nanomolar levels of gangliosides Neu5Acα8Neu5Acα3Galβ4GlcCer, Galβ3GalNAcβ4(Neu5Acα8Neu5Acα3)Galβ4GlcCer (GD1b), Neu5Acα3Galβ3GalNAcβ4(Neu5Acα8Neu5Acα3) Galβ4GlcCer (GT1b) or its oligosaccharide portion induced a rapid and transient activation of Ca 2+ /calmodulin-dependent protein kinase II (CaM-KII) in the subplasmalemma. (elsevierpure.com)
  • Given the structural similarities among different gangliosides on the microtiter plate, anti-GM1 was used as a surrogate standardization. (buhlmannlabs.ch)
  • Because the frequency of metastases in uveal and cutaneous melanoma differs, it is possible that they may express different gangliosides. (duke.edu)
  • Based on these findings, it has been suggested that C jejuni components mimic human gangliosides GM1 and GD1a, and C jejuni infection induces the production of autoantibodies against the gangliosides that are expressed in the peripheral nerves, resulting in the limb weakness seen in GBS. (bmj.com)
  • This study compared anti-C. jejuni IgA, IgG, and IgM antibody levels, as well as the likelihood of testing positive for any of five anti-ganglioside autoantibodies, between animal farm ers and non- farm ers. (cdc.gov)
  • The relationship between anti-C. jejuni antibody levels and detection of anti-ganglioside autoantibodies was also assessed. (cdc.gov)
  • Fisher's Exact Tests and logistic regression were used to compare likelihood of positivity for anti-ganglioside autoantibodies. (cdc.gov)
  • A higher percentage of farm ers (21%) tested positive for anti-ganglioside autoantibodies compared to non- farm ers (9%), but this difference was not statistically significant (p = 0.11). (cdc.gov)
  • There was no significant association between anti-C. jejuni antibody levels and anti-ganglioside autoantibodies. (cdc.gov)
  • In this thesis, the role of gangliosides in glial proliferation, migration and differentiation as well as the regeneration of the olfactory system and myelination were studied using mice lacking enzymes involved in ganglioside biosynthesis. (gla.ac.uk)
  • Role of ganglioside biosynthesis genetic polymorphism in cervical cancer development. (cdc.gov)
  • We show that activation of Neu3 sialidase, also known as Neuraminidase-3, causing conversion of GD1a and GT1b to GM1 ganglioside, is an essential step in regeneration occurring in PNS (sensory) but not CNS (retinal) axons in adult rat. (jneurosci.org)
  • In PNS axons, axotomy activates Neu3 sialidase, increasing the ratio of GM1/GD1a and GM1/GT1b gangliosides immediately after injury in vitro and in vivo . (jneurosci.org)
  • Externally applied sialidase converted GD1a ganglioside to GM1 and rescued axon regeneration in CNS axons and in PNS axons after Neu3 sialidase blockade. (jneurosci.org)
  • Sensitisation of rabbits with bovine brain gangliosides or isolated GM1 produced a replica of axonal GBS. (bmj.com)
  • Differential distribution of major brain gangliosides in the adult mouse central nervous system. (uiowa.edu)
  • These findings suggest that complex gangliosides modulate glial cell function to some extent. (gla.ac.uk)
  • However, since the effects observed were subtle, it is possible that simpler gangliosides are able to compensate for the lack of complex gangliosides. (gla.ac.uk)
  • However, the localisation of sodium channels at the node of Ranvier and potassium channels at juxtaparanode was retarded in GalNAc T -/- mice lacking all complex gangliosides, suggesting that complex gangliosides modulate the formation of nodes of Ranvier. (gla.ac.uk)
  • In addition, GalNAc T -/- mice had significantly lower numbers of NG2 positive early oligodendrocyte progenitors compared to wild-type and Sia T -/- mice, suggesting that complex gangliosides may affect early progenitor differentiation, proliferation or survival. (gla.ac.uk)
  • In this paper, we examine Neu3 sialidase action on axonal gangliosides and resultant axon regeneration, and we assess whether these mechanisms help to explain the different regeneration capacities of CNS and PNS axons. (jneurosci.org)
  • There is now good evidence that gangliosides or similar components trigger the development of axonal GBS. (bmj.com)
  • Axonal density and myelination were unaffected in ganglioside knockout mice. (gla.ac.uk)
  • Matreya now offers a number of high purity deuterated gangliosides which are ideal for mass spectrometry studies. (matreya.com)
  • The development of refined methods for the analysis of gangliosides by high-performance liquid chromatography coupled to mass spectrometry (HPLC-MS) has supported research with qualitative and quantitative data. (chimia.ch)
  • In this article, the strengths of hydrophilic interaction liquid chromatography (HILIC) for baseline separation of gangliosides, including two structural isomers, and their structural characterization by tandem mass spectrometry are demonstrated. (chimia.ch)
  • Thus, obtained results demonstrate that the negative ion mode MS, MS 2 and MS 3 spectra provided sufficient information for the determination of molecular weights, oligosaccharide sequences and ceramide structures, and indicate that the AP-MALDI-QIT-TOF mass spectrometry keeping analytes atmospheric conditions with MS n switching is quite useful and convenient for structural analyses of various types of sialic acid-containing GSLs, gangliosides. (fujita-hu.ac.jp)
  • GM1 ganglioside induces phosphorylation and activation of Trk and Erk in brain. (bio-conferences.org)
  • B4GALNT1 induces angiogenesis, anchorage independence growth and motility, and promotes tumorigenesis in melanoma by induction of ganglioside GM2/GD2. (nih.gov)
  • Gangliosides, which are complex lipids with a sugar chain, are present in all eukaryotic cell membranes and comprise lipid rafts. (bvsalud.org)
  • Scholars@Duke publication: Variations in the ganglioside profile of uveal melanoma correlate with cytologic heterogeneity. (duke.edu)
  • Gangliosides may play an important role in the proliferation and spread of human malignant melanoma. (duke.edu)
  • We analyzed the ganglioside profiles of primary uveal melanoma in 14 cases and of cutaneous melanoma metastasis in 19 cases. (duke.edu)
  • These results suggest that ganglioside expression of uveal melanoma is associated with host immune responses to the tumor. (duke.edu)
  • Furthermore, the low metastatic capacity of uveal melanoma, in contrast to the high metastatic rate of cutaneous melanoma, may be a result of its differentiated ganglioside expression, which is strikingly similar to that of normal melanocytes. (duke.edu)
  • Scholars@Duke publication: Cell surface reactive human monoclonal antibody directed to human melanoma-associated gangliosides. (duke.edu)
  • The preferential binding of 7c11.e8 to melanoma tissues and the reactivity with two of the major melanoma gangliosides (GM3 and GD3) suggest that 7c11.e8 may provide a useful reagent for diagnosis and therapy of malignant melanoma. (duke.edu)
  • and gangliosides (anti-GQ1b IgG) were negative. (cdc.gov)
  • The functions of gangliosides as specific determinants suggest its important role in the growth and differentiation of tissues as well as in carcinogenesis. (wikipedia.org)
  • It has been found that tumor formation can induce the synthesis of a new complement of ganglioside, and very low concentrations of a specific ganglioside can induce differentiation of cultured neuronal tumor cells. (wikipedia.org)
  • Natural and semisynthetic gangliosides are considered possible therapeutics for neurodegenerative disorders. (wikipedia.org)
  • Alteration of the ganglioside profile is associated with various neurodegenerative diseases and there is indication that gangliosides are involved in the pathogenesis of Parkinson's and Huntington's disease. (chimia.ch)
  • Gangliosides have also been shown to have neuroprotective actions and have been considered as candidates for the treatment of several neurodegenerative disorders. (gla.ac.uk)
  • This gene provides instructions for making an enzyme called GM3 synthase, which carries out a chemical reaction that is the first step in the production of molecules called gangliosides. (medlineplus.gov)
  • Without this enzyme, cells cannot produce gangliosides normally. (medlineplus.gov)
  • Membrane redistribution of gangliosides and glycosylphosphatidylinositol-anchored proteins in brain tissue sections under conditions of lipid raft isolation. (uiowa.edu)
  • Effects of detergents on the redistribution of gangliosides and GPI-anchored proteins in brain tissue sections. (uiowa.edu)
  • A ganglioside is a molecule composed of a glycosphingolipid (ceramide and oligosaccharide) with one or more sialic acids (e.g. (wikipedia.org)
  • The oligosaccharide groups on gangliosides extend well beyond the surfaces of the cell membranes, and act as distinguishing surface markers that can serve as specific determinants in cellular recognition and cell-to-cell communication. (wikipedia.org)
  • The filopodia formation induced by the gangliosides may have a physiological relevance because long-term exposure of hippocampal neurons to GT1b oligosaccharide induced advanced dendritogenesis. (elsevierpure.com)
  • The display by DH1, of a surface glycan that mimics the terminal trisaccharide portion of disialosyl-containing gangliosides, provides strong evidence for its involvement in the development of Fisher syndrome. (diva-portal.org)
  • These results suggest that oligosaccharides of the gangliosides serve as potential regulators of the filopodia formation in neuronal cells by triggering the activation of CaM-KII followed by cdc42 up-regulation via a cell surface receptor-like component. (elsevierpure.com)
  • Ganglioside GD2 identifies breast cancer stem cells and promotes tumorigenesis[J]. J Clin Invest, 2012, 122: 2066-2078. (magtechjournal.com)
  • The optimal conditions for EIA were examined by using chicken anti-GM 1 ganglioside (GM 1 ) serum and the final procedure was as follows. (elsevierpure.com)
  • Mutations in genes coding for these enzymes leads to the accumulation of partially broken down gangliosides in lysosomes, which results in a group of diseases called gangliosidosis. (wikipedia.org)
  • 3 A deficiency in the enzymes responsible for metabolizing gangliosides causes toxic levels of ganglioside accumulation which is indicative of several lysosomal storage diseases, including GM 1 gangliosidosis, GM 2 gangliosidosis, Tay-Sachs disease, and Sandhoff disease. (matreya.com)
  • Pharmacological blocking of Neu3 sialidase, downregulation of Neu3 with siRNA, or depleting gangliosides all inhibit PNS regeneration. (jneurosci.org)
  • Regeneration of the olfactory system in ganglioside knockout mice was similar to that of wild-type mice. (gla.ac.uk)
  • Gangliosides are nerve growth, stable biofilm development and regeneration of the nervous system. (galaklc.com)
  • The name ganglioside was first applied by the German scientist Ernst Klenk in 1942 to lipids newly isolated from ganglion cells of the brain. (wikipedia.org)
  • Gangliosides are present and concentrated on cell surfaces, with the two hydrocarbon chains of the ceramide moiety embedded in the plasma membrane and the oligosaccharides located on the extracellular surface, where they present points of recognition for extracellular molecules or surfaces of neighboring cells. (wikipedia.org)
  • Gangliosides are a family of conjugates consisting of a polar sialoglycan head group and a hydrophobic ceramide tail. (chimia.ch)
  • Etiology of vision loss in ganglioside GM3 synthase deficiency. (medlineplus.gov)
  • Differential roles of gangliosides in malignant properties of melanomas[J]. PLoS One, 2018, 13: e0206881. (magtechjournal.com)
  • Cholera Tetanus Botulism Leprosy Obesity, where inadequate ganglioside expression in mediobasal hypothalamic neurons deregulates neuronal leptin and insulin signaling. (wikipedia.org)
  • Recently, gangliosides have been found to be highly important molecules in immunology. (wikipedia.org)
  • Conclusions Ganglioside GD2 is a potential marker for LCSCs to identify cancer stem cells, providing a novel strategy of GD2-targeted lung cancer immunothearapy. (magtechjournal.com)
  • Nevertheless, without an anti-ganglioside antibody assay, in Korea AMAN is frequently misdiagnosed as AIDP by single electrophysiological studies. (nih.gov)
  • Anti-NeuAc-sialosylparagloboside and anti-NeuGc-sialosylparagloboside sera showed a high specificity for the homologous ganglioside. (elsevierpure.com)
  • Gangliosides and gangliosidoses: principles of molecular and metabolic pathogenesis. (bio-conferences.org)
  • Background Ganglioside GM3 mediates adipocyte insulin level of resistance, but the part of GM3 in diabetic wound recovery, a major reason behind morbidity, is unclear. (ap26113.com)
  • Results indicate that the administration of the drug under both acute and chronic conditions brings about characteristic changes in the sialoglycoproteins and ganglioside content in all the subcellular fractions. (caluniv.ac.in)
  • Once you receive your Ganglioside IgG Antibody Panel test results, your physician might advise you with corrective measures if they are not in the normal range. (bajajfinservhealth.in)
  • Thus, the ganglioside/CaM-KII signal plays a role in modulating dendritic morphogenesis by inducing cdc42-mediated actin reorganization. (elsevierpure.com)
  • Therefore, in this study, we elucidated the role of ganglioside GM3 in mast cells and skin inflammation. (bvsalud.org)
  • Targeting O-Acetyl-GD2 ganglioside for cancer immunotherapy[J]. J Immunol Res, 2017, 2017: 5604891. (magtechjournal.com)