An internal standard for the quantification of isobutyryl-L-carnitine
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Unlabeled Version(s)
37103Isobutyryl-L-carnitine (chloride)
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Isobutyryl-L-carnitine-d3 (chloride)

Item No. 26576

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
(R)-3-carboxy-2-(isobutyryloxy)-N,N-dimethyl-N-(methyl-d3)propan-1-aminium, monochloride
Synonyms
  • CAR 4:0-d3
  • C4:0 Carnitine-d3
  • L-Carnitine isobutyryl ester-d3
  • L-Isobutyrylcarnitine-d3
Molecular Formula
C11H19D3NO4 • Cl
Formula Weight
270.8
Purity
≥99% deuterated forms (d1-d3)
Formulation (Request formulation change)
A crystalline solid
Solubility(Learn about Variance in Solubility)
DMF: 20 mg/mlDMSO: 20 mg/mlEthanol: 25 mg/mlPBS (pH 7.2): 10 mg/ml
SMILES
OC(C[C@@H](OC(C(C)C)=O)C[N+](C)(C)C([2H])([2H])[2H])=O.[Cl-]
InChi Code
InChI=1S/C11H21NO4.ClH/c1-8(2)11(15)16-9(6-10(13)14)7-12(3,4)5;/h8-9H,6-7H2,1-5H3;1H/t9-;/m1./s1/i3D3;
InChi Key
FWUACOYFRJEMMP-WINMCZQPSA-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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    1 mg

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    Product Description

    Isobutyryl-L-carnitine-d3 is intended for use as an internal standard for the quantification of isobutyryl-L-carnitine by GC- or LC-MS. Isobutyryl-L-carnitine is a natural 4-carbon acylcarnitine that is involved in fatty acid oxidation and organic acid metabolism.1 Elevated levels of isobutyryl-L-carnitine are associated with isobutyryl-CoA dehydrogenase deficiency.2,3

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Turer, A.T., Stevens, R.D., Bain, J.R., et al. Metabolomic profiling reveals distinct patterns of myocardial substrate use in humans with coronary artery disease or left ventricular dysfunction during surgical ischemia/reperfusion. Circulation 119(13), 1736-1746 (2009).

    2. Koeberl, D.D., Young, S.P., Gregersen, N., et al. Rare disorders of metabolism with elevated butyryl- and isobutyryl-carnitine detected by tandem mass spectrometry newborn screening. Pediatr. Res. 54(2), 219-223 (2003).

    3. Rinaldo, P., Cowan, T.M., and Matern, D. Acylcarnitine profile analysis. Genet. Med. 10(2), 151-156 (2008).