Carnitine is a quaternary amine that occurs naturally in most mammalian tissue. It is present in relatively high concentrations in skeletal muscle and heart where it is involved in regulating energy metabolism. It shifts glucose metabolism from glycolysis to glycogen storage and enhances the transport of long chain fatty acids into the mitochondria where they are oxidized for energy production. Carnitine is biosynthesized from lysine. It might be associated with the energy production from branched chain amino acids. L-Carnitine hydrochloride has been used to study ergothioneine transporter SLC22A4 and carnitine transporter SLC22A5. It has also been used to allow the entry of palmitic acid into the mitochondria. References: Nunes V, Niinikoski H. Lysinuric Protein Intolerance. 2006 Dec 21 [updated 2018 Apr 12]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from .gov/books/NBK1361/ PubMed PMID: 20301535.

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CAS：6645-46-1