Carnitine deficiency-induced cardiomyopathy
Carnitine deficiency-induced cardiomyopathy
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DOI：
10.1023/a:1006826620218
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发表时间：
1998-03-01
影响因子：
4.3
通讯作者：
Paulson, DJ
中科院分区：
文献类型：
--
作者：
Paulson, DJ
临床和动物研究的结果表明，短期内中度继发性肉毒碱缺乏症，本身并没有对心脏收缩功能产生重大影响，尽管底物氧化可能会改变。然而，随着肉毒碱缺乏症的持续时间的延长，心脏内发生变化，可能导致收缩性能受损，特别是在高工作负荷下。在这一点上，负责心脏抑制的机制是不确定的。我们推测，在底物代谢的改变所产生的肉毒碱缺乏的状态下，在高负荷条件下，导致心脏收缩能力受损的ATP生产不足的结果。肉毒碱缺乏也可能引起正常心脏收缩功能和代谢所需的关键酶的基因表达的一些变化。
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.