Formulation: A crystalline solid
Formal Name: (R)-3-carboxy-2-(heptadecanoyloxy)-N,N-dimethyl-N-(methyl-d3)propan-1-aminium, monochloride
Purity: ≥95%
Formula Markup: C24H45D3NO4 • Cl
Formula Weight: 453,1
Shelf life (days): 1460
Notes: Heptadecanoyl-L-carnitine-d3 is intended for use as an internal standard for the quantification of heptadecanoyl-L-carnitine by GC- or LC-MS. Heptadecanoyl-L-carnitine is a long-chain acylcarnitine composed of heptadecanoic acid (Item No. 19722) and L-carnitine (Item No. 21489). Levels of heptadecanoyl-L-carnitine are increased in dried blood spots from neonates with propionic or methylmalonic acidemias, inborn errors of metabolism characterized by a deficiency in the activity of propionyl-CoA carboxylase or L-methylmalonyl-CoA mutase or its cofactor 5-deoxyadenosylcobalamin, respectively.{50369}