Mitochondrial quality control by the Pink1/Parkin system

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• 作者：Cornelia Rüb ; Anne Wilkening ; Wolfgang Voos
• 关键词：Pink1 ; Parkin ; Mitochondria ; Organellar quality control ; Mitophagy
• 刊名：Cell and Tissue Research
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：367
• 期：1
• 页码：111-123
• 全文大小：
• 刊物类别：Biomedical and Life Sciences
• 刊物主题：Human Genetics; Proteomics; Molecular Medicine;
• 出版者：Springer Berlin Heidelberg
• ISSN：1432-0878
• 卷排序：367

文摘

Mitochondrial dysfunction represents a prominent pathological feature in many neurodegenerative diseases, particularly in Parkinson’s disease (PD). Mutations in the genes encoding the proteins Pink1 and Parkin have been identified as genetic risk factors in familiar cases of PD. Research during the last decade has identified both proteins as crucial components of an organellar quality control system that contributes to the maintenance of mitochondrial function in healthy cells. The Pink1/Parkin system acts as a sensor for mitochondrial quality and is activated, in particular, after the loss of the electric potential across the inner mitochondrial membrane. Pink1 molecules accumulate at the surface of damaged mitochondria to recruit and activate Parkin, which, in turn, elicits a signaling pathway eventually leading to the autophagic removal of the damaged organelles. This review summarizes recent advances in our knowledge of the functional role of the Pink1/Parkin system in preventing the accumulation of damaged mitochondria by mitophagy.