Disorders in ketolysis

Proteins (5)

Protein	Synonyms	Taxonomy
Hydroxymethylglutaryl-CoA synthase, cytoplasmic	HMG-CoA synthase; EC 2.3.3.10; 3-hydroxy-3-methylglutaryl coenzyme A synthase	Homo sapiens (human)
Acetyl-CoA acetyltransferase, mitochondrial	EC 2.3.1.9; Acetoacetyl-CoA thiolase; T2	Homo sapiens (human)
Acetyl-CoA acetyltransferase, cytosolic	EC 2.3.1.9; Acetyl-CoA transferase-like protein; Cytosolic acetoacetyl-CoA thiolase	Homo sapiens (human)
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial	EC 2.8.3.5; 3-oxoacid CoA-transferase 1; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; SCOT-s	Homo sapiens (human)
D-beta-hydroxybutyrate dehydrogenase, mitochondrial	EC 1.1.1.30; 3-hydroxybutyrate dehydrogenase; BDH; Short chain dehydrogenase/reductase family 9C member 1	Homo sapiens (human)

Compound	Description
Succinate
3-hydroxybutyric acid	BUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.
3-hydroxy-3-methylglutaryl-coenzyme a	RN given refers to cpd without isomeric designation
acetoacetate
succinyl-coenzyme a
acetyl coenzyme a	Acetyl 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