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.

Isovalerylcarnitine chloride (Standard) is a product of L-leucine catabolism and a selective calpain activator that induces apoptosis; it is associated with Isovaleric Acidemia and is one of the markers of this disease.Isovalerylcarnitine chloride (Standard) can be used for quantitative analysis, study of metabolic pathways, mechanisms of apoptosis, and diagnosis of related diseases.

3-Hydroxyisovalerylcarnitine (Standard) is an acylcarnitine involved in fatty acid metabolism, and is a biomarker of biotin deficiency, with concentrations that are significantly elevated in biotin deficiency. As a standard, 3-Hydroxyisovalerylcarnitine (Standard) can be used for quantitative analysis, study of metabolic pathways and diagnosis of related diseases.

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].