isovalerylcarnitine

Isovalerylcarnitine (3-methylbutyrylcarnitine) is a small molecule compound produced by the catabolism of L-leucine and the accumulation of isovaleric acid. Isovalerylcarnitine is also a selective and effective calpain activator that can promote cell apoptosis. Isovalerylcarnitine is related to isovaleric acidemia and can be used as a marker of isovaleric acidemia.

3-Hydroxyisovalerylcarnitine is a carnitine derivative that may serve as a novel biomarker for biotin deficiency in humans, with its concentration increasing in such conditions. Biotin deficiency reduces the activity of biotin-dependent 3-methylcrotonyl-CoA carboxylase, hindering the conversion of 3-methylcrotonyl-CoA into 3-methylglutaconyl-CoA, and impairing the leucine catabolism pathway, resulting in elevated plasma 3-Hydroxyisovalerylcarnitine concentrations [1].