An internal standard for the quantification of 2-methylbutyryl-L-carnitine
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Unlabeled Version(s)
308182-Methylbutyryl-L-carnitine
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2-Methylbutyryl-L-carnitine-d3 (chloride)

Item No. 26572

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
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 5 mg/ml solution in ethanol
Solubility(Learn about Variance in Solubility)
DMF: 15 mg/mlDMSO: 20 mg/mlEthanol: 25 mg/mlPBS (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
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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Certificates of Analysis & Batch Specific Data

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    500 µg

    $90

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    1 mg

    $168

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    5 mg

    $696

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    Product Description

    2-Methylbutyryl-L-carnitine-d3 is intended for use as an internal standard for the quantification of 2-methylbutyryl-L-carnitine (Item No. 30818) by GC- or LC-MS. 2-Methylbutyryl-L-carnitine is a naturally occurring acylcarnitine that is produced via L-isoleucine metabolism.1 2-Methylbutyryl-L-carnitine levels decrease in OVCAR-8 and adriamycin-resistant OVCAR-8 ovarian cancer cells undergoing ferroptosis induced by erastin (Item No. 17754).2 Plasma levels of 2-methylbutyryl-L-carnitine are elevated in patients with non-alcoholic steatohepatitis (NASH).3 Elevated levels of 2-methylbutyryl-L-carnitine are associated with 2-methylbutyryl-CoA dehydrogenase deficiency (2-MBCDD), also known as short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency.4

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Gibson, K.M., Burlingame, T.G., Hogema, B., et al. 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: A new inborn error of L-isoleucine metabolism. Pediatr. Res. 47(6), 830-833 (2000).

    2. Kirkwood-Donelson, K.I., Jarmusch, A.K., Bortner, C.D., et al. Metabolic consequences of erastin-induced ferroptosis in human ovarian cancer cells: An untargeted metabolomics study. Front. Mol. Biosci. 11, 1520876 (2025).

    3. Kalhan, S.C., Guo, L., Edmison, J., et al. Plasma metabolomic profile in nonalcoholic fatty liver disease. Metabolism 60(3), 404-413 (2011).

    4. Van Calcar, S.C., Baker, M.W., Williams, P., et al. Prevalence and mutation analysis of short/branched chain acyl-CoA dehydrogenase deficiency (SBCADD) detected on newborn screening in Wisconsin. Mol. Genet. Metab. 110(1-2), 111-115 (2013).