De la levure aux maladies neurodégénératives : dix ans d'exploration des pathologies de la dynamique mitochondriale
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De la levure aux maladies neurodégénératives : dix ans d'exploration des pathologies de la dynamique mitochondriale. / Lenaers, Guy; Amati-Bonneau, Patrizia; Delettre, Cécile; Chevrollier, Arnaud; Verny, Christophe; Miléa, Dan; Procaccio, Vincent; Bonneau, Dominique; Hamel, Christian; Reynier, Pascal.
I: Medecine Sciences, Bind 26, Nr. 10, 01.10.2010, s. 836-41.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - De la levure aux maladies neurodégénératives : dix ans d'exploration des pathologies de la dynamique mitochondriale
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
Y1 - 2010/10/1
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 - http://dx.doi.org/10.1051/medsci/20102610836
DO - http://dx.doi.org/10.1051/medsci/20102610836
M3 - Tidsskriftartikel
VL - 26
SP - 836
EP - 841
JO - Medecine Sciences
JF - Medecine Sciences
SN - 0767-0974
IS - 10
ER -
ID: 34112856