Page last updated: 2024-10-06

GABA-Transaminase Deficiency

Proteins (9)

ProteinSynonymsTaxonomy
Glutamate decarboxylase 1EC 4.1.1.15; 67 kDa glutamic acid decarboxylase; GAD-67; Glutamate decarboxylase 67 kDa isoformHomo sapiens (human)
4-aminobutyrate aminotransferase, mitochondrialEC 2.6.1.19; (S)-3-amino-2-methylpropionate transaminase; 2.6.1.22; GABA aminotransferase; GABA-AT; Gamma-amino-N-butyrate transaminase; GABA transaminase; GABA-T; L-AIBATHomo sapiens (human)
Dihydropyrimidine dehydrogenase [NADP(+)]DHPDHase; DPD; EC 1.3.1.2; Dihydrothymine dehydrogenase; Dihydrouracil dehydrogenaseHomo sapiens (human)
Aldehyde dehydrogenase, mitochondrialEC 1.2.1.3; ALDH class 2; ALDH-E2; ALDHIHomo sapiens (human)
Beta-ureidopropionaseEC 3.5.1.6; BUP-1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolaseHomo sapiens (human)
Membrane primary amine oxidaseEC 1.4.3.21; Copper amine oxidase; HPAO; Semicarbazide-sensitive amine oxidase; SSAO; Vascular adhesion protein 1; VAP-1Homo sapiens (human)
DihydropyrimidinaseDHP; DHPase; EC 3.5.2.2; Dihydropyrimidine amidohydrolase; HydantoinaseHomo sapiens (human)
Beta-Ala-His dipeptidaseEC 3.4.13.20; CNDP dipeptidase 1; Carnosine dipeptidase 1; Glutamate carboxypeptidase-like protein 2; Serum carnosinaseHomo sapiens (human)
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrialMMSDH; Malonate-semialdehyde dehydrogenase [acylating]; EC 1.2.1.18; EC 1.2.1.27; Aldehyde dehydrogenase family 6 member A1Homo sapiens (human)

Compounds (24)

CompoundDescription
hydronium ion
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
glutamic acidA non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
NADH
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
Coenzyme A
n-carbamoyl-beta-alanineRN given refers to parent cpd
uracilOne of four nucleotide bases in the nucleic acid RNA.
beta-alanineAn amino acid formed in vivo by the degradation of dihydrouracil and carnosine. Since neuronal uptake and neuronal receptor sensitivity to beta-alanine have been demonstrated, the compound may be a false transmitter replacing GAMMA-AMINOBUTYRIC ACID. A rare genetic disorder, hyper-beta-alaninemia, has been reported.
trimethylenediamineRN given refers to parent cpd; structure
alpha-ketoglutaric acid
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)
histidineAn essential amino acid that is required for the production of HISTAMINE.
dihydrouracil
3-methylhistidinemarker for myofibrillar-protein breakdown; RN given refers to (L)-isomer
3-aminopropionaldehydestructure given in first source
malonic semialdehydeRN given refers to parent cpd; structure given in first source
anserineA dipeptide containing BETA-ALANINE.
carnosineA naturally occurring dipeptide neuropeptide found in muscles.
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.
creolinfrom refined coal tar oils