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The PINK1/Parkin pathway: a mitochondrial quality control system?

Whitworth, A.J. and Pallanck, L.J. (2009) The PINK1/Parkin pathway: a mitochondrial quality control system? Journal of Bioenergetics and Biomembranes, 41 (6). pp. 499-503. ISSN 0145-479X

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Significant insight into the mechanisms that contribute to dopaminergic neurodegeneration in Parkinson disease has been gained from the analysis of genes linked to rare heritable forms of parkinsonism such as PINK1 and parkin, loss-of-function mutations of which cause autosomal recessive parkinsonism. PINK1 encodes a mitochondrially targeted Ser/Thr kinase and parkin encodes a ubiquitin-protein ligase. Functional studies of PINK1 and Parkin in animal and cellular model systems have shown that both proteins play important roles in maintaining mitochondrial integrity. Genetic studies of PINK1 and Parkin orthologs in flies have shown that PINK1 acts upstream from Parkin in a common pathway that appears to regulate mitochondrial morphology. Mitochondrial morphology is regulated by mitochondrial fission and fusion-promoting proteins, and is important in a variety of contexts, including mitochondrial trafficking and mitochondrial quality control. In particular, mitochondrial fission appears to promote the segregation of terminally dysfunctional mitochondria for degradation in the lysosome through a process termed mitophagy. Recent work has shown that Parkin promotes the degradation of dysfunctional mitochondria in vertebrate cell culture. Here we postulate a model whereby the PINK1/Parkin pathway regulates mitochondrial dynamics in an effort to promote the turnover of damaged mitochondria.

Item Type: Article
Copyright, Publisher and Additional Information: © 2009 Springer. This is an author produced version of a paper subsequently published in Journal of Bioenergetics and Biomembranes. Uploaded in accordance with the publisher's self-archiving policy.
Keywords: Mitophagy; Autophagy; Turnover
Institution: The University of Sheffield
Academic Units: The University of Sheffield > Faculty of Science (Sheffield) > School of Biological Sciences (Sheffield) > Department of Biomedical Science (Sheffield) > Centre for Developmental Genetics (Sheffield)
The University of Sheffield > University of Sheffield Research Centres and Institutes > Centre for Developmental Genetics (Sheffield)
Depositing User: Miss Anthea Tucker
Date Deposited: 29 Jan 2010 10:42
Last Modified: 08 Feb 2013 16:59
Published Version: http://dx.doi.org/10.1007/s10863-009-9253-3
Status: Published
Publisher: Springer
Refereed: Yes
Identification Number: 10.1007/s10863-009-9253-3
URI: http://eprints.whiterose.ac.uk/id/eprint/10333

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