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 (IBDH) activity and to support diagnosis of related metabolic deficiencies.¹ It is also applied in metabolic flux analysis to study CrAT-mediated transport of intermediates for lipid biosynthesis. Additionally, it serves as a metabolic probe to examine how stressors such as hypoxia alter lipid composition, including reduced mmBCFA diversity in obese adipose tissue.²
Other available forms
