Systemic Carnitine Deficiency

J. D. McGarry, D. W. Foster

Research output: Contribution to journalEditorialpeer-review

31 Scopus citations


Carnitine was discovered in 1905, and its structure was established in 1927. It is a relatively simple molecule that is synthesized from lysine, with terminal methyl groups donated by S-adenosylmethionine. From studies in lower species it is thought that the immediate precursor γ-butyrobetaine can be formed in many tissues (e.g., kidney, muscle, and heart), but that hydroxylation to carnitine occurs to an important extent only in liver, as shown below: Carnitine synthesized in the hepatocyte is released into plasma for transfer to peripheral tissues, where it is taken up against a concentration gradient by means of a transport system that. . .

Original languageEnglish (US)
Pages (from-to)1413-1415
Number of pages3
JournalNew England Journal of Medicine
Issue number24
StatePublished - Dec 11 1980

ASJC Scopus subject areas

  • General Medicine


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