Article
Up-regulation of mitochondrial uncoupling protein 3 reveals an early muscular metabolic defect in amyotrophic lateral sclerosis.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Nov 2003
Dupuis Luc, di Scala Franck, Rene Frédérique, de Tapia Marc, Oudart Hugues, Pradat Pierre-François, Meininger Vincent, Loeffler Jean-Philippe
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting primarily motor neurons. Growing evidence suggests a mitochondrial defect in ALS. The precise molecular mechanisms underlying those defects are unknown. We studied the expression of mitochondrial uncoupling proteins (UCPs), key regulators of mitochondrial functions, in tissues from a mouse model of ALS (SOD1 G86R transgenic mice) and...
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