VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease

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• 作者：Xing Guo^a ; Xin Qi^a ; ^b ; ^{xxq38@case.edu}
• 关键词：Valosin-containing protein ; Mitochondria ; Protein degradation ; Huntington's disease
• 刊名：Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：1863
• 期：2
• 页码：552-559
• 全文大小：1206 K
• 卷排序：1863

文摘

Mutant Htt promotes mitochondrial outer membrane protein MCL1 degradation via the UPS. Mutant Htt recruits VCP to the mitochondria, where VCP mediates MCL1 degradation. UBXD1 is a cofactor of VCP to facilitate MCL1 degradation in HD models.