Structure via PubChem · Public domain (PubChem)
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
In the Vinony graph
Within Vinony's link graph, Isovaleryl-CoA is referenced by 102 other articles, and connects out to digital object identifier, International Standard Serial Number and chemical formula.
It sits within the topics Chembox image size set and Thioesters of coenzyme A.
Its subject is documented across 10 Wikipedia language editions.
Chemical data
- Formula
- C26H44N7O17P3S
- Molecular weight
- 851.7 g/mol
- IUPAC name
- S-[2-[3-[[(2R)-4-[[[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-phosphonooxyoxolan-2-yl]methoxy-hydroxyphosphoryl]oxy-hydroxyphosphoryl]oxy-2-hydroxy-3,3-dimethylbutanoyl]amino]propanoylamino]ethyl] 3-methylbutanethioate
- 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=C(N=CN=C32)N)O)OP(=O)(O)O)O
- InChIKey
- UYVZIWWBJMYRCD-ZMHDXICWSA-N
- XLogP
- -4.3
- Polar surface area
- 389 Ų
- H-bond donors
- 9
- H-bond acceptors
- 22
- Formal charge
- 0
via PubChem
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
via Wikidata · CC0
~3 min read
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.