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

TitreDe la levure aux maladies neurodégénératives : Dix ans d’exploration des pathologies de la dynamique mitochondriale
Type de publicationArticle de revue
AuteurLenaers, Guy, Amati-Bonneau, Patrizia , Delettre, Cécile, Chevrollier, Arnaud , Verny, Christophe, Milea, Dan , Procaccio, Vincent , Bonneau, Dominique , Hamel, Christian, Reynier, Pascal
EditeurEDP Sciences
TypeArticle scientifique dans une revue à comité de lecture
Année2010
LangueAnglais
Date2010
Numéro10
Pagination836 - 841
Volume26
Titre de la revueMédecine/sciences
ISSN0767-0974
Mots-clésAxons/physiology, GTP Phosphohydrolases/genetics, Humans, Mitochondria/physiology, Mitochondrial Diseases/genetics/physiopathology, Neurodegenerative Diseases/genetics/physiopathology, Optic Nerve Diseases/genetics, Oxidative Phosphorylation, Yeasts/physiology
Résumé en anglais

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.

URL de la noticehttp://okina.univ-angers.fr/publications/ua8210
DOI10.1051/medsci/20102610836
Lien vers le document

http://dx.doi.org/10.1051/medsci/20102610836

Titre abrégéMed Sci