Evaluating protein cross-linking as a therapeutic strategy to stabilize SOD1 variants in a mouse model of familial ALS.

Evaluating protein cross-linking as a therapeutic strategy to stabilize SOD1 variants in a mouse model of familial ALS.

Mutations in the gene encoding Cu-Zn superoxide dismutase 1 (SOD1) cause a subset of familial amyotrophic lateral sclerosis (fALS) cases. A shared effect of these mutations is that SOD1, which is normally a stable dimer, dissociates into toxic monomers that seed toxic aggregates. Considerable resear...

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Main Authors: Md Amin Hossain, Richa Sarin, Daniel P Donnelly, Brandon C Miller, Alexandra Weiss, Luke McAlary, Svetlana V Antonyuk, Joseph P Salisbury, Jakal Amin, Jeremy B Conway, Samantha S Watson, Jenifer N Winters, Yu Xu, Novera Alam, Rutali R Brahme, Haneyeh Shahbazian, Durgalakshmi Sivasankar, Swathi Padmakumar, Aziza Sattarova, Aparna C Ponmudiyan, Tanvi Gawde, David E Verrill, Wensheng Yang, Sunanda Kannapadi, Leigh D Plant, Jared R Auclair, Lee Makowski, Gregory A Petsko, Dagmar Ringe, Nathalie Y R Agar, David J Greenblatt, Mary Jo Ondrechen, Yunqiu Chen, Justin J Yerbury, Roman Manetsch, S Samar Hasnain, Robert H Brown, Jeffrey N Agar
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2024-01-01
Series:PLoS Biology
Online Access:https://journals.plos.org/plosbiology/article/file?id=10.1371/journal.pbio.3002462&type=printable

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