Isovaleryl Coenzyme A, Sodium salt

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 starter unit in the biosynthesis of iso-fatty acids in organisms such as myxobacteria, where it is elongated with malonyl-CoA to form lipids like 13-methyltetradecanoic acid. It is also used to study alternative metabolic pathways, as it can be synthesized from acetyl-CoA via the mevalonate pathway when amino acid degradation is impaired.¹ Clinically, isovaleryl-CoA serves as a biomarker for isovaleric acidemia (IVA), detected through elevated derivatives in blood and urine. Management includes conversion into non-toxic conjugates (isovalerylglycine, isovalerylcarnitine) via glycine and carnitine supplementation. It is also used in assays to measure isovaleryl-CoA dehydrogenase activity and to study genotype–phenotype relationships in organic acidemias.² These roles highlight isovaleryl-CoA as both a biosynthetic intermediate and an indicator of metabolic health.¹ ²

 

Other available forms

 

Isovaleryl Coenzyme A, Free acid