Paulson D J
Department of Physiology, Midwestern University, Downers Grove, Illinois 60515, USA.
Mol Cell Biochem. 1998 Mar;180(1-2):33-41.
The results of clinical and animal studies suggest that a short term period of moderate secondary carnitine deficiency, in and of itself, does not have a major effect on the cardiac contractile function, although substrate oxidation may be altered. However, with longer durations of carnitine deficiency, alterations occur within the heart that may result in impaired contractile performance, particularly at high workloads. At this point, the mechanisms responsible for the cardiac depression are uncertain. We hypothesize that the alterations in substrate metabolism produced by the carnitine deficient state results in inadequate ATP production under high workload conditions which result in impaired cardiac contractile performance. Carnitine deficiency may also induce a number of changes in gene expression of key enzymes required for normal cardiac contractile function and metabolism.
临床和动物研究结果表明，短期内中度继发性肉碱缺乏本身对心脏收缩功能没有重大影响，尽管底物氧化可能会改变。然而，随着肉碱缺乏时间延长，心脏内会发生变化，这可能导致收缩功能受损，尤其是在高负荷工作时。此时，导致心脏抑制的机制尚不确定。我们推测，肉碱缺乏状态引起的底物代谢改变会导致在高负荷条件下ATP生成不足，从而导致心脏收缩功能受损。肉碱缺乏还可能诱导正常心脏收缩功能和代谢所需关键酶的基因表达发生一系列变化。
