2-Methylbutyryl-<wbr/>L-carnitine-d<sub>3</sub> (chloride)

2-Methylbutyryl-L-carnitine-d3 (chloride)

CAT N°: 26572
Price:

From 122.00 103.70

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}

Territorial Availability: Available through Bertin Technologies only in France

  • Synonyms
    • (2R)-3-carboxy-N,N-dimethyl-N-(methyl-d3)-2-((2-methylbutanoyl)oxy)propan-1-aminium, monochloride
  • Correlated keywords
    • deuterated deuterium methyl-butyryl butyroyl nonalcoholic 2MBCDD coenzyme A acylCoA
  • Product Overview:
    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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