Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis.

  title={Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis.},
  author={Julie D. Atkin and Manal A. Farg and Kai Ying Soo and Adam K. Walker and Mark A. Halloran and Bradley J. Turner and Phillip Nagley and Malcolm K. Horne},
  journal={Journal of neurochemistry},
  volume={129 1},
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 transport is linked to ER stress, Golgi fragmentation and axonal transport and we… CONTINUE READING