Page last updated: 2024-10-06

Disorders in ketone body synthesis

Proteins (4)

ProteinSynonymsTaxonomy
Hydroxymethylglutaryl-CoA synthase, mitochondrialHMG-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)
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 (11)

CompoundDescription
hydronium ion
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
NADH
acetoneA colorless liquid used as a solvent and an antiseptic. It is one of the ketone bodies produced during ketoacidosis.
3-hydroxybutyric acidBUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.
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)
leucineAn essential branched-chain amino acid important for hemoglobin formation.
acetoacetate
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