Isovaleryl Coenzyme A, Free acid
CAS No.: 6244-91-3
Synonym(s): Isovaleryl Coenzyme A, IV-CoA, Isovaleryl-CoA, 3-Methylbutyryl-CoAAvailability: 2-3 weeks
Isovaleryl-CoA is a branched-chain acyl-CoA derived from leucine catabolism that serves as a critical starter unit for iso-fatty acid biosynthesis in myxobacteria.¹ In humans, it is processed by isovaleryl-CoA dehydrogenase, an enzyme whose deficiency leads to isovaleric acidemia and the accumulation of toxic derivatives.¹ ² Certain myxobacteria can also produce isovaleryl-CoA through an alternative pathway branching from mevalonate-dependent isoprenoid biosynthesis when standard leucine degradation is impaired.¹
Applications
Isovaleryl-CoA is a key intermediate in leucine catabolism and serves as a starter unit for iso-fatty acid biosynthesis in organisms such as myxobacteria. It can also be produced from acetyl-CoA through an alternative mevalonate pathway when branched-chain amino acid degradation is impaired.¹ Clinically, isovaleryl-CoA metabolism is central to isovaleric acidemia (IVA), and its accumulation and derivatives help support diagnosis.² Isovaleryl-CoA is used as a substrate in isovaleryl-CoA dehydrogenase (IVD) activity assays and studies of leucine catabolism and IVA.³ ⁴ It is also used to investigate coenzyme B₁₂-dependent interconversion with pivalyl-CoA and branched-chain fatty acid biosynthesis.⁵ ⁶ Isovaleryl-CoA might serve as an HPLC or LC-MS reference standard.⁷
Other available forms
