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De la levure aux maladies neurodégénératives: Dix ans d’exploration des pathologies de la dynamique mitochondriale

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Title: De la levure aux maladies neurodégénératives: Dix ans d’exploration des pathologies de la dynamique mitochondriale
Authors: Lenaers, Guy, Amati-Bonneau, Patrizia, Delettre, Cécile, Chevrollier, Arnaud, Verny, Christophe, Milea, Dan, Procaccio, Vincent, Bonneau, Dominique, Hamel, Christian, Reynier, Pascal
Contributors: Univ Angers, Okina
Source: médecine/sciences. 26:836-841
Publisher Information: EDP Sciences, 2010.
Publication Year: 2010
Subject Terms: Optic Nerve Diseases/genetics, [SDV] Life Sciences [q-bio], 0301 basic medicine, 0303 health sciences, 03 medical and health sciences, Neurodegenerative Diseases/genetics/physiopathology, Axons/physiology, Humans, Mitochondria/physiology, Mitochondrial Diseases/genetics/physiopathology, Yeasts/physiology, GTP Phosphohydrolases/genetics, Oxidative Phosphorylation
Description: Ten years ago, OPA1 was identified as the major gene responsible for hereditary optic nerve degeneration, evidencing the first defect in mitochondrial network dynamics as the princeps pathophysiological mechanism in a mitochondriopathy. Later, alterations in other genes involved in mitochondrial fusion or fission, such as MFN2, DRP1 and GDAP1, were also associated with inherited neurological diseases, mainly affecting peripheral nerves. More recently, altered mitochondrial plasticity was also demonstrated in common age-related neurodegenerative disorders, as Alzheimer and Parkinson diseases, thus substantiating the critical role of mitochondrial dynamics in neurons as a key element governing the efficiency of oxidative respiration and its distribution along the axons.
Document Type: Article
ISSN: 1958-5381
0767-0974
DOI: 10.1051/medsci/20102610836
Access URL: https://www.medecinesciences.org/articles/medsci/pdf/2010/09/medsci20102610p836.pdf
Accession Number: edsair.doi.dedup.....eb6b04f6ec477da17e005381e90bc3e5
Database: OpenAIRE
Description
Abstract:Ten years ago, OPA1 was identified as the major gene responsible for hereditary optic nerve degeneration, evidencing the first defect in mitochondrial network dynamics as the princeps pathophysiological mechanism in a mitochondriopathy. Later, alterations in other genes involved in mitochondrial fusion or fission, such as MFN2, DRP1 and GDAP1, were also associated with inherited neurological diseases, mainly affecting peripheral nerves. More recently, altered mitochondrial plasticity was also demonstrated in common age-related neurodegenerative disorders, as Alzheimer and Parkinson diseases, thus substantiating the critical role of mitochondrial dynamics in neurons as a key element governing the efficiency of oxidative respiration and its distribution along the axons.
ISSN:19585381
07670974
DOI:10.1051/medsci/20102610836