Page last updated: 2024-10-06

Succinyl CoA: 3-Ketoacid CoA Transferase Deficiency

Proteins (4)

ProteinSynonymsTaxonomy
Acetyl-CoA acetyltransferase, mitochondrialEC 2.3.1.9; Acetoacetyl-CoA thiolase; T2Homo sapiens (human)
Hydroxymethylglutaryl-CoA lyase, mitochondrialHL; HMG-CoA lyase; EC 4.1.3.4; 3-hydroxy-3-methylglutarate-CoA lyaseHomo sapiens (human)
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrialEC 2.8.3.5; 3-oxoacid CoA-transferase 1; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; SCOT-sHomo sapiens (human)
D-beta-hydroxybutyrate dehydrogenase, mitochondrialEC 1.1.1.30; 3-hydroxybutyrate dehydrogenase; BDH; Short chain dehydrogenase/reductase family 9C member 1Homo sapiens (human)

Compounds (10)

CompoundDescription
succinic acidA water-soluble, colorless crystal with an acid taste that is used as a chemical intermediate, in medicine, the manufacture of lacquers, and to make perfume esters. It is also used in foods as a sequestrant, buffer, and a neutralizing agent. (Hawley's Condensed Chemical Dictionary, 12th ed, p1099; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1851)
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
NADH
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)
Coenzyme A
acetoneA colorless liquid used as a solvent and an antiseptic. It is one of the ketone bodies produced during ketoacidosis.
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)
3-hydroxybutyric acidBUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.
acetoacetic 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.