Isovaleryl-CoA
Sign in to saveAlso known as isovaleryl-coenzyme a
Isovaleryl-CoA (also known as 3-methylbutyryl-CoA) is a metabolic intermediate formed during the catabolism of the branched-chain amino acid, leucine. It is a short-chain acyl-CoA thioester that plays a key role in mitochondrial energy metabolism. The compound is converted into 3-methylcrotonyl-CoA by the enzyme isovaleryl-CoA dehydrogenase (IVD), a flavoprotein that catalyzes the third step in the leucine degradation pathway. Deficiency of this enzyme activity results in the accumulation of isovaleryl-CoA and related metabolites, leading to a rare autosomal recessive disorder known as isovale
Wikidata facts
- Mass
- 851.173
Show 3 more facts
- chemical formula
- C₂₆H₄₄N₇O₁₇P₃S
- canonical SMILES
- CC(C)CC(=O)SCCNC(=O)CCNC(=O)C(C(C)(C)COP(=O)(O)OP(=O)(O)OCC1C(C(C(O1)N2C=NC3=C2N=CN=C3N)O)OP(=O)(O)O)O
- isomeric SMILES
- CC(C)CC(=O)SCCNC(=O)CCNC(=O)[C@@H](C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@@H]1[C@H]([C@H]([C@@H](O1)N2C=NC3=C2N=CN=C3N)O)OP(=O)(O)O)O
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Encyclopedic overview
2 sectionsContents
- Catalysis of isovaleryl-CoA dehydrogenase
- References
{{chembox | Verifiedfields = changed | Watchedfields = changed | verifiedrevid = 432474661 | ImageFile = Isovaleryl coenzyme A.svg | ImageClass = skin-invert | ImageSize = 300px | IUPACName = 3′-O-Phosphonoadenosine 5′-[(3R)-3-hydroxy-2,2-dimethyl-4-{[3-({2-[(3-methylbutanoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-4-oxobutyl dihydrogen diphosphate] | PIN = O1-{[(2R,3S,4R,5R)-5-(6-Amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methyl} O3-[(3R)-3-hydroxy-2,2-dimethyl-4-{[3-({2-[(3-methylbutanoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-4-oxobutyl] dihydrogen diphosphate | OtherNames = | Section1 = | Section2 = | Section3 = }}
Isovaleryl-CoA (also known as 3-methylbutyryl-CoA) is a metabolic intermediate formed during the catabolism of the branched-chain amino acid, leucine. It is a short-chain acyl-CoA thioester that plays a key role in mitochondrial energy metabolism. The compound is converted into 3-methylcrotonyl-CoA by the enzyme isovaleryl-CoA dehydrogenase (IVD), a flavoprotein that catalyzes the third step in the leucine degradation pathway. Deficiency of this enzyme activity results in the accumulation of isovaleryl-CoA and related metabolites, leading to a rare autosomal recessive disorder known as isovaleric acidemia, characterized by metabolic crises, developmental delays, and a distinctive odor due to isovaleric acid buildup. The metabolism of isovaleryl-CoA is vital for proper amino acid utilization and energy homeostasis in humans.
Excerpted from Wikipedia’s “Isovaleryl-CoA” article, available under the CC BY-SA 4.0 licence.