肉碱可降低丙酸代谢紊乱患者的空腹生酮作用。

Carnitine reduces fasting ketogenesis in patients with disorders of propionate metabolism.

作者信息

Wolff J A, Carroll J E, Prodanos C, Haas R, Nyhan W L

出版信息

Lancet. 1986 Feb 8;1(8476):289-91. doi: 10.1016/s0140-6736(86)90826-3.

Abstract

Patients with disorders of propionate metabolism have low plasma levels of free carnitine and excrete higher than normal quantities of esterified carnitine. The response to a 19 h fast was assessed as a physiological index of carnitine deficiency. In patients with propionic acidaemia and methylmalonic acidaemia a substantial ketogenesis developed in response to fasting. Supplementation with L-carnitine significantly reduced this ketogenic response.

摘要

患有丙酸代谢紊乱的患者血浆中游离肉碱水平较低，且酯化肉碱的排泄量高于正常水平。将对19小时禁食的反应作为肉碱缺乏的生理指标进行评估。在患有丙酸血症和甲基丙二酸血症的患者中，禁食会引发大量酮体生成。补充左旋肉碱可显著降低这种生酮反应。