Article
Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis.
Journal of neurochemistry - 1 Apr 2014
Atkin Julie D, Farg Manal A, Soo Kai Ying, Walker Adam K, Halloran Mark, Turner Bradley J, Nagley Phillip, Horne Malcolm K
Abstract excerpt
Cu/Zn-superoxide dismutase is misfolded in familial and sporadic amyotrophic lateral sclerosis, but it is not clear how this triggers endoplasmic reticulum (ER) stress or other pathogenic processes. Here, we demonstrate that mutant SOD1 (mSOD1) is predominantly found in the cytoplasm in neuronal cells. Furthermore, we show that mSOD1 inhibits secretory protein transport from the ER to Golgi apparatus. ER-Golgi...
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