Formulation: A solution in ethanol
Formal Name: (2R)-3-carboxy-N,N-dimethyl-N-(methyl-d3)-2-((2-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: 2-Methylbutyryl-L-carnitine-d3 is intended for use as an internal standard for the quantification of 2-methylbutyryl-L-carnitine by GC- or LC-MS. 2-Methylbutyryl-carnitine is a naturally occurring acylcarnitine that is produced via L-isoleucine metabolism.{42603} Plasma levels of 2-methylbutyryl-carnitine are elevated in patients with non-alcoholic steatohepatitis (NASH).{42604} Elevated levels of 2-methylbutyryl-carnitine are associated with 2-methylbutyryl-CoA dehydrogenase deficiency (2-MBCDD), also known as short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency.{42605}
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