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Papers In Press, published online ahead of print October 21, 2002
Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466-8550
Corresponding Author: sobueg{at}med.nagoya-u.ac.jp
The mutations in superoxide dismutase 1 (SOD1) cause approximately 20% of familial amyotrophic lateral sclerosis (FALS) cases. A toxic and gain function has been considered as the cause of the disease, but its molecular mechanism remains uncertain. To determine whether the subcellular localization of mutant SOD1 is crucial to mutant SOD1-mediated cell death, we produced neuronal cell models with accumulation of SOD1 in each subcellular fraction/organelle such as the cytosol, nucleus, endoplasmic reticulum, and mitochondria. Here we showed that the localization of mutant SOD1 in the mitochondria triggered the release of mitochondrial cytochrome c followed by the activation of caspase cascade and induced neuronal cell death without cytoplasmic mutant SOD1-aggregate formation. Nuclear and endoplasmic reticulum localization of mutant SOD1 did not induce cell death. These results suggest that the localization of mutant SOD1 in the mitochondria is critical in the pathogenesis of mutant SOD1-associated FALS.
J. Biol. Chem, 10.1074/jbc.M209356200
Submitted on September 12, 2002
Revised on October 21, 2002
Accepted on October 21, 2002
Mitochondrial localization of mutant SOD1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis
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