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Protein inclusions rich in mutant Cu,Zn superoxide dismutase (SOD1) have been found in tissues from patients with familial amyotrophic lateral sclerosis (ALS). Here, the mouse motor neuron-like NSC-34 cell line transiently transfected with human SOD1 G93A fused to enhanced green fluorescent protein exhibited aggregates contrary to cells overexpressing wild-type human SOD1. The aggregates were...
A familial form of the neurodegenerative disease amyotrophic lateral sclerosis (ALS), is caused by dominant mutations in the cytosolic Cu,Zn superoxide dismutase (SOD1). There has been evidence for secretion of SOD1, by an unknown mechanism. In this work stable mouse motor neuron-like NSC-34 cells overexpressing human SOD1 wild-type hSOD1 wt (NSC-34/hSOD1 wt ) and mutant hSOD1 ...
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