| Identification | Back Directory | [Name]
1-Propanaminium, 3-carboxy-2-hydroxy-N,N,N-tri(methyl-d3)-, chloride (1:1), (2R)- | [CAS]
2687961-04-0 | [Synonyms]
| [Molecular Formula]
C7H16ClNO3 | [MOL File]
2687961-04-0.mol | [Molecular Weight]
197.66 |
| Hazard Information | Back Directory | [Uses]
L-Carnitine-d9 (chloride)e is the deuterium labeled L-Carnitine chloride. L-Carnitine chloride, a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine chloride functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine chloride is an antioxidant. L-Carnitine chloride can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3]. | [Biological Activity]
L-Carnitine-d9 is intended for use as an internal standard for the quantification of L-carnitine by GC- or LC-MS. L-Carnitine is a conditionally essential nutrient.1 It is obtained from dietary sources or through the metabolism of lysine and methionine.2,3 L-Carnitine facilitates the transport of long-chain fatty acids into the mitochondrial matrix for β-oxidation, has other diverse roles on metabolism, and is involved in the maintenance of coenzyme A stores. Plasma and/or tissue levels of L-carnitine are decreased in primary L-carnitine deficiency, a disorder characterized by impaired fatty acid oxidation, with symptoms varying depending on whether it is systemic or muscle-specific.1 Serum and tissue levels of L-carnitine are also reduced in secondary L-carnitine deficiencies caused by a variety of hereditary defects or acquired disorders. | [References]
1.Seim, H., Eichler, K., and Kleber, H.-P.L(-)-Carnitine and its precursor, γ-butyrobetaineNutraceuticals in Health and Disease Prevention217-256(2001)
2.Vaz, F.M., and Wanders, R.J.A.Carnitine biosynthesis in mammalsBiochem. J.361(Pt 3)417-429(2002)
3.Fu, L., Huang, M., and Chen, S.Primary carnitine deficiency and cardiomyopathyKorean. Circ. J.43(12)785-792(2013)
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