The Redox Activity of Protein Disulfide Isomerase Inhibits ALS Phenotypes in Cellular and Zebrafish Models

Summary: Pathological forms of TAR DNA-binding protein 43 (TDP-43) are present in almost all cases of amyotrophic lateral sclerosis (ALS), and 20% of familial ALS cases are due to mutations in superoxide dismutase 1 (SOD1). Redox regulation is critical to maintain cellular homeostasis, although how...

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Main Authors: Sonam Parakh, Sina Shadfar, Emma R. Perri, Audrey M.G. Ragagnin, Claudia V. Piattoni, Mariela B. Fogolín, Kristy C. Yuan, Hamideh Shahheydari, Emily K. Don, Collen J. Thomas, Yuning Hong, Marcelo A. Comini, Angela S. Laird, Damian M. Spencer, Julie D. Atkin
Format: Article
Language:English
Published: Elsevier 2020-05-01
Series:iScience
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Online Access:http://www.sciencedirect.com/science/article/pii/S2589004220302820