TY - JOUR
T1 - Systemic Carnitine Deficiency
AU - McGarry, J. D.
AU - Foster, D. W.
PY - 1980/12/11
Y1 - 1980/12/11
N2 - 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. . .
AB - 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. . .
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U2 - 10.1056/NEJM198012113032410
DO - 10.1056/NEJM198012113032410
M3 - Editorial
C2 - 7432389
AN - SCOPUS:0019177965
SN - 0028-4793
VL - 303
SP - 1413
EP - 1415
JO - New England Journal of Medicine
JF - New England Journal of Medicine
IS - 24
ER -