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)