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

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. Plasma levels of 2-methylbutyryl-carnitine are elevated in patients with non-alcoholic steatohepatitis (NASH). 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.Formal Name: (2R)-3-carboxy-N,N-dimethyl-N-(methyl-d3)-2-((2-methylbutanoyl)oxy)propan-1-aminium, monochloride. Synonyms: CAR 5:0-d3, C5:0 Carnitine-d3, L-Carnitine 2-methylbutyroyl ester-d3, L-Carnitine 2-methylbutyryl ester-d3, 2-Methylbutyroylcarnitine-d3, 2-Methylbutyrylcarnitine-d3. Molecular Formula: C12H21D3NO4 . Cl. Formula Weight: 284.8. Purity: >99% deuterated forms (d1-d3). Formulation: (Request formulation change), A solution in ethanol. Solubility: DMF: 15 mg/ml, DMSO: 20 mg/ml, Ethanol: 25 mg/ml, PBS (pH 7.2): 10 mg/ml. SMILES: OC(C[C@H](C[N+](C([2H])([2H])[2H])(C)C)OC(C(C)CC)=O)=O.[Cl-]. InChi Code: InChI=1S/C12H23NO4.ClH/c1-6-9(2)12(16)17-10(7-11(14)15)8-13(3,4)5,/h9-10H,6-8H2,1-5H3,1H/t9?,10-,/m1./s1/i3D3,. InChi Key: NJQTVSRXHPLZGN-VRSOTZOVSA-N.