Formulation: A solid
Formal Name: (R)-3-carboxy-N,N-dimethyl-N-(methyl-d3)-2-((3-methylbutanoyl)oxy)propan-1-aminium, monochloride
Purity: ≥99% deuterated forms (d1-d3)
Formula Markup: C12H21D3NO4 • Cl
Formula Weight: 284,8
Shelf life (days): 730
Notes: Isovaleryl-L-carnitine-d3 is intended for use as an internal standard for the quantification of isovaleryl-L-carnitine (Item No. 26555) by GC- or LC-MS. Isovaleryl-L-carnitine is a naturally occurring acylcarnitine that is formed via metabolism of L-leucine.{43591} It increases survival and decreases apoptosis in hepatocyte growth factor-deprived murine C2.8 hepatocytes when used at a concentration of 1 mM.{43592} 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.{43593} Increased levels of isovaleryl carnitine are associated with isovaleryl-CoA dehydrogenase deficiency (isovaleric acidemia).{32028}
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