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Each pathway is unique and responsible for degradation of distinctive cytosolic material. The ubiquitin\u2010proteasome system and autophagy (chaperone\u2010mediated, macro, micro and organelle specific) act synergistically to maintain proteostasis. Defects in this equilibrium can be deleterious at cellular and organism level, giving rise to various disease states. Dysfunction of quality control pathways are implicated in neurodegenerative diseases and appear particularly important in <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>arkinson's disease and the lysosomal storage disorders. Neurodegeneration resulting from impaired degradation of ubiquitinated proteins and \u03b1\u2010synuclein is often accompanied by mitochondrial dysfunction. Mitochondria have evolved to control a diverse number of processes, including cellular energy production, calcium signalling and apoptosis, and like every other organelle within the cell, they must be \u2018recycled.\u2019 Failure to do so is potentially lethal as these once indispensible organelles become destructive, leaking reactive oxygen species and activating the intrinsic cell death pathway. This process is paramount in neurons which have an absolute dependence on mitochondrial oxidative phosphorylation as they cannot up\u2010regulate glycolysis. As such, mitochondrial bioenergetic failure can underpin neural death and neurodegenerative disease. In this review, we discuss the links between cellular quality control and neurodegenerative diseases associated with mitochondrial dysfunction, with particular attention to the emerging links between <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>arkinson's and <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>aucher diseases in which defective quality control is a defining factor.<\/jats:p><\/jats:sec><jats:sec><jats:title>Linked Articles<\/jats:title><jats:p>This article is part of a themed issue on Mitochondrial Pharmacology: Energy, Injury &amp; Beyond. To view the other articles in this issue visit <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/dx.doi.org\/10.1111\/bph.2014.171.issue-8\">http:\/\/dx.doi.org\/10.1111\/bph.2014.171.issue\u20108<\/jats:ext-link><\/jats:p><\/jats:sec>","DOI":"10.1111\/bph.12453","type":"journal-article","created":{"date-parts":[[2013,10,12]],"date-time":"2013-10-12T02:20:50Z","timestamp":1381544450000},"page":"1958-1972","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":68,"title":["Quality control gone wrong: mitochondria, lysosomal storage disorders and neurodegeneration"],"prefix":"10.1111","volume":"171","author":[{"given":"L D","family":"Osellame","sequence":"first","affiliation":[{"name":"Department of Cell and Developmental Biology and UCL Consortium for Mitochondrial Research University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M 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