• Free Radic. Biol. Med. · Nov 2016

    Review

    Mechanisms of mitophagy: PINK1, Parkin, USP30 and beyond.

    • Baris Bingol and Morgan Sheng.
    • Department of Neuroscience, Genentech Inc, South San Francisco, CA 94080, USA. Electronic address: bingol.baris@gene.com.
    • Free Radic. Biol. Med. 2016 Nov 1; 100: 210-222.

    AbstractMitochondrial quality control is central for maintaining a healthy population of mitochondria. Two Parkinson's disease genes, mitochondrial kinase PINK1 and ubiquitin ligase Parkin, degrade damaged mitochondria though mitophagy. In this pathway, PINK1 senses mitochondrial damage and activates Parkin by phosphorylating Parkin and ubiquitin. Activated Parkin then builds ubiquitin chains on damaged mitochondria to tag them for degradation in lysosomes. USP30 deubiquitinase acts as a brake on mitophagy by opposing Parkin-mediated ubiquitination. Human genetic data point to a role for mitophagy defects in neurodegenerative diseases. This review highlights the molecular mechanisms of the mitophagy pathway and the recent advances in the understanding of mitophagy in vivo.Copyright © 2016 Elsevier Inc. All rights reserved.

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