Isovaleryl-L-carnitine-d3 is intended for use as an internal standard for the quantification of isovaleryl-L-carnitine by GC- or LC-MS. Isovaleryl-L-carnitine is a naturally occurring acylcarnitine that is formed via metabolism of L-leucine.1 It increases survival and decreases apoptosis in hepatocyte growth factor-deprived murine C2.8 hepatocytes when used at a concentration of 1 mM.2 Isovaleryl-L-carnitine inhibits amino acid deprivation-induced proteolysis and autophagy in isolated perfused rat liver when used at concentrations of 77 and 100 μM, respectively.3 Increased levels of isovaleryl carnitine are associated with isovaleryl-CoA dehydrogenase deficiency (isovaleric acidemia).4
1.Cavazza, C.L-carnitine derivatives as therapeutical agents for treating myopathies and neuronal degeneration and for inhibiting proteolysis(1993) 2.Revoltella, R.P., Dal Canto, B., Caracciolo, L., et al.L-carnitine and some of its analogs delay the onset of apoptotic cell death initiated in murine C2.8 hepatocytic cells after hepatocyte growth factor deprivationBiochim. Biophys. Acta1224(3)333-341(1994) 3.Miotto, G., Venerando, R., Khurana, K.K., et al.Control of hepatic proteolysis by leucine and isovaleryl-L-carnitine through a common locus. Evidence for a possible mechanism of recognition at the plasma membraneJ. Biol. Chem.267(31)22066-22072(1992) 4.Rinaldo, P., Cowan, T.M., and Matern, D.Acylcarnitine profile analysisGenet.Med.10(2)151-156(2008)