Hepatic ketogenesis and muscle carnitine deficiency
Hepatic ketogenesis and muscle carnitine deficiency
复制标题
肝生酮和肌肉肉碱缺乏
DOI：
10.1212/wnl.29.6.780
复制
发表时间：
1979
期刊：
影响因子：
9.9
通讯作者：
M. Rimoldi
中科院分区：
文献类型：
--
作者：
S. Didonato;F. Cornelio;G. Storchi;M. Rimoldi
血浆游离肉毒碱和酮体的水平已被发现在没有肌肉疾病的禁食个体中呈反比波动。循环短链酰基-肉毒碱β-羟基丁酸水平。一名患有脂质沉积性肌病和肌肉肉毒碱缺乏症的患者和他的两个女儿在禁食时发生了过度的生酮。患者肝脏中的总肉毒碱含量正常，但游离肉毒碱减少到50%，总酯化肉毒碱是对照组平均值的四倍。在这种情况下，肌肉肉毒碱含量降低可能是由于慢性肝生酮，排出肌肉肉毒碱。或者，肌肉肉毒碱含量减少可能会引发肝生酮。
The levels of plasma free carnitine and ketone bodies have been found to fluctuate inversely in fasting individuals without muscle disease. Circulating short-chain acyl-carnitines paralleled β-hydroxybutyrate levels. A patient with lipid storage myopathy and muscle carnitine deficiency, and his two daughters, developed exaggerated ketogenesis on fasting. The content of total carnitines in the patient's liver was normal, but free carnitine was reduced to 50 percent, and total esterified carnitines were four times greater than the mean value for the controls. The decreased muscle carnitine content in this case may have resulted from chronic hepatic ketogenesis, draining muscle carnitine. Alternatively, decreased muscle carnitine content may have initiated hepatic ketogenesis.