Formulation: A solid
Formal Name: 3-carboxy-N,N-dimethyl-N-(methyl-d3)-2-((3-methylbutanoyl)oxy)propan-1-aminium, monochloride
Purity: ≥99% deuterated forms (d1-d3)
Formula Markup: C12H15D3NO4 • Cl
Formula Weight: 278,7
Shelf life (days): 730
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}