Succinate dehydrogenase - Assembly, regulation and role in human disease.

Succinate dehydrogenase - Assembly, regulation and role in human disease.
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DOI:
10.1016/j.mito.2010.03.001
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发表时间:
2010-06
期刊:
影响因子:
4.4
通讯作者:
Schiffman, Joshua D.
Schiffman, Joshua D.
中科院分区:
生物学3区
文献类型:
--
作者:
Rutter, Jared;Winge, Dennis R.;Schiffman, Joshua D.

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琥珀酸脱氢酶(或电子传递链复合物II)一直是重点但重要的复兴的主题。这种复合物,这是线粒体呼吸复合物中研究最少的,由于其在人类疾病中的作用的发现而重新引起了人们的兴趣。在这种高度的审查下,琥珀酸脱氢酶复合物已被证明是一个迷人的机器,其调节和组装需要开始被发现的其他因素。这些因子和复合物本身的结构亚基的突变会导致各种人类疾病。SDH突变的发病机制开始被理解。
Succinate dehydrogenase (or Electron Transport Chain Complex II) has been the subject of a focused but significant renaissance. This complex, which has been the least studied of the mitochondrial respiratory complexes has seen renewed interest due to the discovery of its role in human disease. Under this heightened scrutiny, the succinate dehydrogenase complex has proven to be a fascinating machine, whose regulation and assembly requires additional factors that are beginning to be discovered. Mutations in these factors and in the structural subunits of the complex itself cause a variety of human diseases. The mechanisms underlying the pathogenesis of SDH mutations is beginning to be understood.
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