Disorders in ketone body synthesis

Proteins (4)

Protein	Synonyms	Taxonomy
Hydroxymethylglutaryl-CoA synthase, mitochondrial	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)
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
hydronium ion
Carbon Dioxide	A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
NADH
acetone	A colorless liquid used as a solvent and an antiseptic. It is one of the ketone bodies produced during ketoacidosis.
3-hydroxybutyric acid	BUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.
NAD	A 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)
leucine	An essential branched-chain amino acid important for hemoglobin formation.
acetoacetate
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