Mutant SOD1 accumulation in sensory neurons does not associate with endoplasmic reticulum stress features: Implications for differential vulnerability of sensory and motor neurons to SOD1 toxicity.

Abstract excerpt

Mutations in Cu/Zn-superoxide dismutase (SOD1) cause familial amyotrophic lateral sclerosis (ALS). Previous papers showed that mutant SOD1 accumulates and undergoes misfolding in motor neurons and that the specific interaction of mutant SOD1 with derlin-1 leads to endoplasmic reticulum (ER) stress and activation of the unfolded protein response (UPR). Because evidence shows that mutant SOD1 expression also...

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