TY - JOUR
T1 - Ketogenic response to fasting in human carnitine deficiencies
AU - Di Donato, S.
AU - Peluchetti, D.
AU - Rimoldi, M.
AU - Bertagnolio, B.
AU - Uziel, G.
AU - Cornelio, F.
PY - 1980/1/31
Y1 - 1980/1/31
N2 - Carnitine and its short-chain and long-chain esters have been determined in muscle and liver of two patients with carnitine-deficient myopathy. Plasma carnitines and ketone bodies were monitored in the two patients during a 38-h fast. The two patients showed different ketogenic responses to the fast. One patient, with carnitine deficiency restricted to skeletal muscle, had exaggerated ketone production on fasting. The other patient, with camitine deficiency in skeletal muscle and liver, had poor ketone body production on fasting. It is assumed that the presence of adequate levels of carnitine in the liver are crucial for the activation of ketogenic machinery in liver.
AB - Carnitine and its short-chain and long-chain esters have been determined in muscle and liver of two patients with carnitine-deficient myopathy. Plasma carnitines and ketone bodies were monitored in the two patients during a 38-h fast. The two patients showed different ketogenic responses to the fast. One patient, with carnitine deficiency restricted to skeletal muscle, had exaggerated ketone production on fasting. The other patient, with camitine deficiency in skeletal muscle and liver, had poor ketone body production on fasting. It is assumed that the presence of adequate levels of carnitine in the liver are crucial for the activation of ketogenic machinery in liver.
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U2 - 10.1016/0009-8981(80)90268-5
DO - 10.1016/0009-8981(80)90268-5
M3 - Article
C2 - 7353309
AN - SCOPUS:0018865232
SN - 0009-8981
VL - 100
SP - 209
EP - 214
JO - Clinica Chimica Acta
JF - Clinica Chimica Acta
IS - 3
ER -