Uses
3-Methylbutyryl-DL-carnitine-d9 (hydrochloride) is the deuterium labeled 3-Methylbutyryl-DL-carnitine hydrochloride[1].
Biological Activity
Isovaleryl-DL-carnitine-d9 is intended for use as an internal standard for the quantification of isovaleryl-DL-carnitine by GC- or LC-MS. Isovaleryl-DL-carnitine is a derivative of carnitine . Increased levels of isovaleryl carnitine are associated with isovaleric acidemia, an inborn error of metabolism characterized by a deficiency in isovaleryl-CoA dehydrogenase.1
References
1.Rinaldo, P., Cowan, T.M., and Matern, D.Acylcarnitine profile analysisGenet.Med.10(2)151-156(2008)
