Isobutyryl-L-carnitine-d3 (chloride) (Synonyms: CAR 4:0-d3, C4:0 Carnitine-d3, L-Carnitine isobutyryl ester-d3, L-Isobutyrylcarnitine-d3) |
| Catalog No.GC47463 |
A neuropeptide with diverse biological activities
Products are for research use only. Not for human use. We do not sell to patients.
Cas No.: N/A
Sample solution is provided at 25 µL, 10mM.
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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
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/reperfusionCirculation119(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 screeningPediatr.Res.54(2)219-223(2003) 3.Rinaldo, P., Cowan, T.M., and Matern, D.Acylcarnitine profile analysisGenet.Med.10(2)151-156(2008)
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