Article
G37R SOD1 mutant alters mitochondrial complex I activity, Ca(2+) uptake and ATP production.
Cell calcium - 1 Apr 2011
Coussee Evelyne, De Smet Patrick, Bogaert Elke, Elens Iris, Van Damme Philip, Willems Peter, Koopman Werner, Van Den Bosch Ludo, Callewaert Geert
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective death of motor neurons. Mutations in Cu/Zn superoxide dismutase-1 (SOD1) cause familial ALS but the molecular mechanisms whereby these mutations induce motor neuron death remain controversial. Here, we show that stable overexpression of mutant human SOD1 (G37R) - but not wild-type SOD1 (wt-SOD1) - in mouse neuroblastoma...
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