De la levure aux maladies neurodégénératives : dix ans d'exploration des pathologies de la dynamique mitochondriale

Guy Lenaers; Patrizia Amati-Bonneau; Cécile Delettre; Arnaud Chevrollier; Christophe Verny; Dan Miléa; Vincent Procaccio; Dominique Bonneau; Christian Hamel; Pascal Reynier

doi:10.1051/medsci/20102610836

De la levure aux maladies neurodégénératives : dix ans d'exploration des pathologies de la dynamique mitochondriale

Guy Lenaers, Patrizia Amati-Bonneau, Cécile Delettre, Arnaud Chevrollier, Christophe Verny, Dan Miléa, Vincent Procaccio, Dominique Bonneau, Christian Hamel, Pascal Reynier

8 Citationer (Scopus)

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.

Bidragets oversatte titel	[From yeast to neurodegenerative diseases: ten years of exploration of mitochondrial dynamic disorders]
Originalsprog	Fransk
Tidsskrift	Medecine Sciences
Vol/bind	26
Udgave nummer	10
Sider (fra-til)	836-41
Antal sider	6
ISSN	0767-0974
DOI	https://doi.org/10.1051/medsci/20102610836
Status	Udgivet - 1 okt. 2010

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10.1051/medsci/20102610836

Citationsformater

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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.",

author = "Guy Lenaers and Patrizia Amati-Bonneau and C{\'e}cile Delettre and Arnaud Chevrollier and Christophe Verny and Dan Mil{\'e}a and Vincent Procaccio and Dominique Bonneau and Christian Hamel and Pascal Reynier",

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AU - Lenaers, Guy

AU - Amati-Bonneau, Patrizia

AU - Delettre, Cécile

AU - Chevrollier, Arnaud

AU - Verny, Christophe

AU - Miléa, Dan

AU - Procaccio, Vincent

AU - Bonneau, Dominique

AU - Hamel, Christian

AU - Reynier, Pascal

PY - 2010/10/1

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N2 - 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.

AB - 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.

U2 - 10.1051/medsci/20102610836

DO - 10.1051/medsci/20102610836

M3 - Tidsskriftartikel

SN - 0767-0974

VL - 26

SP - 836

EP - 841

JO - Medecine Sciences

JF - Medecine Sciences

IS - 10

ER -

De la levure aux maladies neurodégénératives : dix ans d'exploration des pathologies de la dynamique mitochondriale

Abstract

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