Article
Mutant SOD1 alters the motor neuronal transcriptome: implications for familial ALS.
Brain : a journal of neurology - 1 Jul 2005
Kirby Janine, Halligan Eugene, Baptista Melisa J, Allen Simon, Heath Paul R, Holden Hazel, Barber Sian C, Loynes Catherine A, Wood-Allum Clare A, Lunec Joseph, Shaw Pamela J
Abstract excerpt
Familial amyotrophic lateral sclerosis (FALS) is caused, in 20% of cases, by mutations in the Cu/Zn superoxide dismutase gene (SOD1). Although motor neuron injury occurs through a toxic gain of function, the precise mechanism(s) remains unclear. Using an established NSC34 cellular model for SOD1-associated FALS, we investigated the effects of mutant SOD1 specifically in cells modelling the vulnerable cell...
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