Page last updated: 2024-10-06

Disorders in ketolysis

Proteins (5)

ProteinSynonymsTaxonomy
Hydroxymethylglutaryl-CoA synthase, cytoplasmicHMG-CoA synthase; EC 2.3.3.10; 3-hydroxy-3-methylglutaryl coenzyme A synthaseHomo sapiens (human)
Acetyl-CoA acetyltransferase, mitochondrialEC 2.3.1.9; Acetoacetyl-CoA thiolase; T2Homo sapiens (human)
Acetyl-CoA acetyltransferase, cytosolicEC 2.3.1.9; Acetyl-CoA transferase-like protein; Cytosolic acetoacetyl-CoA thiolaseHomo 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 (8)

CompoundDescription
Succinate
3-hydroxybutyric acidBUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.
3-hydroxy-3-methylglutaryl-coenzyme aRN given refers to cpd without isomeric designation
acetoacetate
succinyl-coenzyme a
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.
coenzyme a
acetoacetyl coa