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Biochimie. 2014 May;100:177-83. doi: 10.1016/j.biochi.2013.07.033. Epub 2013 Aug 16.

Mitochondrial quality control in neurodegenerative diseases.

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  • 1INSERM, U1118, Strasbourg F-67085, France; Université de Strasbourg, Fédération de Médecine Translationnelle (FMTS), UMRS1118, Strasbourg F-67085, France. Electronic address:


Mutations causing genetic forms of Parkinson's disease or hereditary neuropathies have been recently shown to affect key molecular players involved in the recycling of defective mitochondria, most notably PARKIN, PINK1, Mitofusin 2 or dynein heavy chain. Interestingly, the same pathways are also indirectly targeted by multiple other mutations involved in familial forms of amyotrophic lateral sclerosis, Huntington's disease or Alzheimer's disease. These recent genetic results strongly reinforce the notion that defective mitochondrial physiology might cause neurodegeneration. Mitochondrial dysfunction has however been observed in virtually every neurodegenerative disease and appears not restricted to the most vulnerable neuronal populations affected by a given disease. Thus, the mechanisms linking defective mitochondrial quality control to death of selective neuronal populations remain to be identified. This review provides an update on the most recent literature on mitochondrial quality control and its impairment during neurodegenerative diseases.


Amyotrophic lateral; Charcot–Marie–Tooth disease; Mitophagy; Parkinson's disease; Sclerosis

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