Notes: Isovaleryl-DL-carnitine-d3 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 (Item Nos. 21489 / 16749). Increased levels of isovaleryl carnitine are associated with isovaleric acidemia, an inborn error of metabolism characterized by a deficiency in isovaleryl-CoA dehydrogenase.{32028}