Page last updated: 2024-10-06

Biotin metabolism, including IMDs

Proteins (9)

ProteinSynonymsTaxonomy
BiotinidaseBiotinase; EC 3.5.1.12Homo sapiens (human)
Acetyl-CoA carboxylase 1ACC1; EC 6.4.1.2; Acetyl-Coenzyme A carboxylase alpha; ACC-alphaHomo sapiens (human)
Propionyl-CoA carboxylase alpha chain, mitochondrialPCCase subunit alpha; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit alphaHomo sapiens (human)
Propionyl-CoA carboxylase beta chain, mitochondrialPCCase subunit beta; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit betaHomo sapiens (human)
Acetyl-CoA carboxylase 2EC 6.4.1.2; ACC-betaHomo sapiens (human)
Biotin--protein ligaseEC 6.3.4.-; Biotin apo-protein ligaseHomo sapiens (human)
Pyruvate carboxylase, mitochondrialEC 6.4.1.1; Pyruvic carboxylase; PCBHomo sapiens (human)
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrialMCCase subunit alpha; EC 6.4.1.4; 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alphaHomo sapiens (human)
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrialMCCase subunit beta; EC 6.4.1.4; 3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit betaHomo sapiens (human)

Compounds (9)

CompoundDescription
biotinA water-soluble, enzyme co-factor present in minute amounts in every living cell. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk.
Pyruvic AcidAn intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
OxaloacetateAn anionic form of oxaloacetic acid.
LeucineAn essential branched-chain amino acid important for hemoglobin formation.
endolysin
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.
Malonyl Coenzyme AA coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.
propionyl-coenzyme ARN given refers to parent cpd
biotinamide