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
{{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.
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