Article
Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 5 Jan 2005
Turner Bradley J, Atkin Julie D, Farg Manal A, Zang Da Wei, Rembach Alan, Lopes Elizabeth C, Patch Justin D, Hill Andrew F, Cheema Surindar S
Abstract excerpt
Mutations in the intracellular metalloenzyme superoxide dismutase 1 (SOD1) are linked to neurotoxicity in familial amyotrophic lateral sclerosis (ALS) by an unclear mechanism. Golgi fragmentation and endoplasmic reticulum stress are early hallmarks of spinal motor neuron pathology in transgenic mice overexpressing mutant SOD1, suggesting that dysfunction of the neuronal secretory pathway may contribute to ALS...
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