【24h】

Mitochondrial dysfunction in acute hyperammonemia.

机译:急性高氨血症的线粒体功能障碍。

获取原文
获取原文并翻译 | 示例
       

摘要

Acute hyperammonemia resulting from congenital urea cycle disorders, Reye syndrome or acute liver failure results in severe neuronal dysfunction, seizures and death. Increasing evidence suggests that acute hyperammonemia results in alterations of mitochondrial and cellular energy function resulting from ammonia-induced inhibition of the tricarboxylic acid cycle enzyme alpha-ketoglutarate dehydrogenase and by activation of the NMDA receptor. Antagonists of this receptor and NOS inhibitors prevent acute ammonia-induced seizures and mortality and prevent acute ammonia-induced changes in mitochondrial calcium homeostasis and cellular energy metabolism. Acute hyperammonemia also results in decreased activities of free radical scavenging enzymes and again, free radical formation due to ammonia exposure is prevented by either NMDA receptor antagonists or NOS inhibitors. Acute hyperammonemia also results in activation of peripheral-type enzymes which are localized on the mitochondrial membranes of astrocytes in the CNS. Activation of these receptors results in mitochondrial swelling and in increased degradation of monoamines, respectively. Alterations of mitochondrial function could contribute to the neuronal dysfunction characteristic of acute hyperammonemic syndromes.
机译:先天性尿素循环异常,雷伊综合征或急性肝衰竭引起的急性高氨血症会导致严重的神经元功能障碍,癫痫发作和死亡。越来越多的证据表明,急性高氨血症会导致线粒体和细胞能量功能的改变,这是由氨诱导的三羧酸循环酶α-酮戊二酸脱氢酶的抑制作用以及NMDA受体的激活引起的。该受体和NOS抑制剂的拮抗剂可防止急性氨诱发的癫痫发作和死亡,并防止急性氨诱发的线粒体钙稳态和细胞能量代谢变化。急性高氨血症还会导致自由基清除酶的活性降低,而且,NMDA受体拮抗剂或NOS抑制剂可防止由于氨暴露而导致自由基形成。急性高氨血症还导致周围型酶的活化,该酶位于CNS中星形胶质细胞的线粒体膜上。这些受体的激活分别导致线粒体肿胀和单胺降解增加。线粒体功能的改变可能导致急性高氨血症综合征的神经元功能异常。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱:kefu@zhangqiaokeyan.com

京公网安备:11010802029741号 ICP备案号:京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有
  • 客服微信

  • 服务号