Page last updated: 2024-10-06

Cerebral organic acidurias, including diseases

Proteins (5)

ProteinSynonymsTaxonomy
Glutaryl-CoA dehydrogenase, mitochondrialGCD; EC 1.3.8.6Homo sapiens (human)
D-2-hydroxyglutarate dehydrogenase, mitochondrialEC 1.1.99.-Homo sapiens (human)
Malate dehydrogenase Homo sapiens (human)
Aminoacylase-1Homo sapiens (human)
Alcohol dehydrogenase iron-containing protein 1 Homo sapiens (human)

Compounds (23)

CompoundDescription
hydronium ion
NADH
1,5-dihydro-fadchromophore component of E coli DNA photolyase
aspartic acidOne of the non-essential amino acids commonly occurring in the L-form. It is found in animals and plants, especially in sugar cane and sugar beets. It may be a neurotransmitter.
tryptophanAn essential amino acid that is necessary for normal growth in infants and for NITROGEN balance in adults. It is a precursor of INDOLE ALKALOIDS in plants. It is a precursor of SEROTONIN (hence its use as an antidepressant and sleep aid). It can be a precursor to NIACIN, albeit inefficiently, in mammals.
flavin-adenine dinucleotideA condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)
2-amino-4-(2-aminoethoxy)butanoic acid, (DL)-isomer
glutaryl-coenzyme a
alpha-hydroxyglutarate, (l)-isomer
3-hydroxy-3-methylglutaryl-coenzyme aRN given refers to cpd without isomeric designation
2-aminoadipic acidA metabolite in the principal biochemical pathway of lysine. It antagonizes neuroexcitatory activity modulated by the glutamate receptor, N-METHYL-D-ASPARTATE; (NMDA).
alpha-ketoglutaric acid
glutaconic acidRN given refers to parent cpd without isomeric designation; structure
D-2-hydroxyglutarate
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
alpha-ketoadipic acid
glutaric acidRN given refers to parent cpd
endolysin
3-hydroxyglutaric acid
acetyl coenzyme aAcetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.
glutaconyl-coenzyme A
n-acetylaspartic acid
coenzyme a