Isobutyryl Coenzyme A, Lithium salt
CAS No.: 103404-95-1
Synonym(s): Isobutyryl Coenzyme A, IB-CoA, 2-Methylpropanoyl-CoA
Availability: 2-3 weeks
Isobutyryl-CoA is a branched-chain acyl-CoA intermediate formed during mitochondrial catabolism of the essential amino acid valine. It is primarily metabolized by isobutyryl-CoA dehydrogenase (IBDH), and deficiency of this enzyme leads to accumulation of isobutyrylcarnitine in tissues and fluids.¹ Beyond catabolism, it functions as a starter unit for the synthesis of monomethyl branched-chain fatty acids (mmBCFAs), such as iso-C16:0. In adipose tissue, carnitine acetyltransferase (CrAT) enables its export from mitochondria to support cytosolic fatty acid elongation.²
Applications
Isobutyryl-CoA is used as a substrate to assess isobutyryl-CoA dehydrogenase (ACAD8) activity and investigate deficiencies affecting valine catabolism.¹ ³ It is also applied in metabolic flux studies of CrAT-mediated intermediate transport and lipid biosynthesis, and to examine metabolic stress effects on lipid composition, including changes in methyl-branched-chain fatty acids (mmBCFAs) in adipose tissue.² Additional applications include studies of histone lysine isobutyrylation, B₁₂-dependent isobutyryl-CoA mutase activity, and the biosynthesis of myxalamids, where isobutyryl-CoA serves as a starter unit.⁴ ⁵ ⁶ Isobutyryl-CoA might be used as an HPLC or LC-MS reference standard.⁷
Other available forms
