Aspartate-Semialdehyde Dehydrogenase
Aspartate Kinase
Homoserine Dehydrogenase
Succinate-Semialdehyde Dehydrogenase
An enzyme that plays a role in the GLUTAMATE and butanoate metabolism pathways by catalyzing the oxidation of succinate semialdehyde to SUCCINATE using NAD+ as a coenzyme. Deficiency of this enzyme, causes 4-hydroxybutyricaciduria, a rare inborn error in the metabolism of the neurotransmitter 4-aminobutyric acid (GABA).
Methylmalonate-Semialdehyde Dehydrogenase (Acylating)
An enzyme that plays a role in the VALINE; LEUCINE; and ISOLEUCINE catabolic pathways by catalyzing the oxidation of 2-methyl-3-oxopropanate to propanoyl-CoA using NAD+ as a coenzyme. Methylmalonate semialdehyde dehydrogenase deficiency is characterized by elevated BETA-ALANINE and 3-hydropropionic acid.
Brain Diseases, Metabolic, Inborn
Brain disorders resulting from inborn metabolic errors, primarily from enzymatic defects which lead to substrate accumulation, product reduction, or increase in toxic metabolites through alternate pathways. The majority of these conditions are familial, however spontaneous mutation may also occur in utero.
4-Aminobutyrate Transaminase
Hydroxybutyrate Dehydrogenase
Aminomuconate-Semialdehyde Dehydrogenase
Sodium Oxybate
Amino Acid Metabolism, Inborn Errors
Aldehyde Dehydrogenase
Aspartate Aminotransferases
Glutamate-5-Semialdehyde Dehydrogenase
Succinates
Adipates
Glutarates
Glutarates are organic compounds, specifically carboxylic acids, that contain a five-carbon chain with two terminal carboxyl groups and a central methyl group, playing a role in various metabolic processes, including the breakdown of certain amino acids. They can also refer to their salts or esters. Please note that this definition is concise and may not cover all aspects of glutarates in depth.