Muscle mitochondrial uncoupling dismantles neuromuscular junction and triggers distal degeneration of motor neurons.

BACKGROUND:Amyotrophic lateral sclerosis (ALS), the most frequent adult onset motor neuron disease, is associated with hypermetabolism linked to defects in muscle mitochondrial energy metabolism such as ATP depletion and increased oxygen consumption. It remains unknown whether muscle abnormalities i...

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Bibliographic Details
Main Authors: Luc Dupuis, Jose-Luis Gonzalez de Aguilar, Andoni Echaniz-Laguna, Judith Eschbach, Frédérique Rene, Hugues Oudart, Benoit Halter, Caroline Huze, Laurent Schaeffer, Frédéric Bouillaud, Jean-Philippe Loeffler
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2009-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC2671839?pdf=render