Inhibition of mitochondrial protein import by mutant huntingtin

被引：0
|
作者：
Hiroko Yano
Sergei V Baranov
Oxana V Baranova
Jinho Kim
Yanchun Pan
Svitlana Yablonska
Diane L Carlisle
Robert J Ferrante
Albert H Kim
Robert M Friedlander
机构：
[1] Neuroapoptosis Laboratory,Department of Neurological Surgery
[2] University of Pittsburgh,Department of Neurological Surgery
[3] Washington University School of Medicine,Department of Neurology
[4] Washington University School of Medicine,Department of Genetics
[5] Washington University School of Medicine,Department of Developmental Biology
[6] Washington University School of Medicine,undefined
[7] University of Pittsburgh Medical Center,undefined
来源：
Nature Neuroscience | 2014年 / 17卷
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学科分类号：
摘要：
Mitochondrial dysfunction has been associated with Huntington's disease. The authors show that mutant huntingtin binds to the mitochondrial import protein TIM23, leading to a deficit in import. Overexpression of TIM23 subunits partially rescued the import defect and subsequent neuronal death.
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页码：822 / 831
页数：9
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