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

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  1. Aldosterone Blockade Added to Renin-Angiotensin System Blockade to Reduce Albuminuria-A Path for Improved Renoprotection?

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  2. Heart murmur and N-terminal pro-brain natriuretic peptide as predictors of death in 2977 consecutive hospitalized patients

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  1. Accuracy of a Deep Learning System for Classification of Papilledema Severity on Ocular Fundus Photographs

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  2. Optic Disc Classification by Deep Learning versus Expert Neuro-Ophthalmologists

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  3. Artificial Intelligence to Detect Papilledema from Ocular Fundus Photographs

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  4. Genetic association study of exfoliation syndrome identifies a protective rare variant at LOXL1 and five new susceptibility loci

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  5. Increased steroidogenesis promotes early-onset and severe vision loss in females with OPA1 dominant optic atrophy

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  • Guy Lenaers
  • Patrizia Amati-Bonneau
  • Cécile Delettre
  • Arnaud Chevrollier
  • Christophe Verny
  • Dan Miléa
  • Vincent Procaccio
  • Dominique Bonneau
  • Christian Hamel
  • Pascal Reynier
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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.
Translated title of the contribution[From yeast to neurodegenerative diseases: ten years of exploration of mitochondrial dynamic disorders]
Original languageFrench
JournalMedecine Sciences
Volume26
Issue number10
Pages (from-to)836-41
Number of pages6
ISSN0767-0974
DOIs
Publication statusPublished - 1 Oct 2010

ID: 31077744