Protein disulfide isomerase in ALS mouse glia links protein misfolding with NADPH oxidase-catalyzed superoxide production.

@article{Jaronen2013ProteinDI,
  title={Protein disulfide isomerase in ALS mouse glia links protein misfolding with NADPH oxidase-catalyzed superoxide production.},
  author={Merja Jaronen and Piia Vehvil{\"a}inen and Tarja Malm and Velta Keksa-Goldsteine and Eveliina Pollari and Piia K. Valonen and Jari Koistinaho and Gundars Goldsteins},
  journal={Human molecular genetics},
  year={2013},
  volume={22 4},
  pages={646-55}
}
Protein disulfide isomerase (PDI) is an oxidoreductase assisting oxidative protein folding in the endoplasmic reticulum of all types of cells, including neurons and glia. In neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS), up-regulation of PDI is an important part of unfolded protein response (UPR) that is thought to represent an adaption reaction and thereby protect the neurons. Importantly, studies on animal models of familial ALS with mutant Cu/Zn superoxide… CONTINUE READING

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