A single copy transgenic mutant FUS strain reproduces age-dependent ALS phenotypes in


Journal

microPublication biology
ISSN: 2578-9430
Titre abrégé: MicroPubl Biol
Pays: United States
ID NLM: 101759238

Informations de publication

Date de publication:
2021
Historique:
received: 03 08 2021
revised: 31 08 2021
accepted: 15 09 2021
entrez: 27 9 2021
pubmed: 28 9 2021
medline: 28 9 2021
Statut: epublish

Résumé

Mutations in the human DNA/RNA binding protein FUS are associated with amyotrophic lateral sclerosis and frontotemporal lobar degeneration, including some aggressive and juvenile onset forms. Cytoplasmic inclusions of human FUS proteins are observed in various neurodegenerative disorders, such as Huntington's disease or spinocerebellar ataxia, suggesting that FUS proteinopathy may be a key player in neurodegeneration. To better understand the pathogenic mechanisms of FUS, we created single copy transgenic

Identifiants

pubmed: 34568776
doi: 10.17912/micropub.biology.000473
pmc: PMC8459179
doi:

Types de publication

Journal Article

Langues

eng

Informations de copyright

Copyright: © 2021 by the authors.

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Auteurs

Audrey Labarre (A)

Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), Montreal, Canada.
Department of Neuroscience, Université de Montréal, Montreal, Canada.

Gilles Tossing (G)

Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), Montreal, Canada.
Department of Neuroscience, Université de Montréal, Montreal, Canada.

Claudia Maios (C)

Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), Montreal, Canada.

James J Doyle (JJ)

Division of Experimental Medicine, Faculty of Medicine, McGill University, Montreal, Canada.
Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), Montreal, Canada.
Metabolic Disorders and Complications, Research Institute of the McGill University Health Centre , Montreal, Canada.

J Alex Parker (JA)

Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), Montreal, Canada.
Department of Neuroscience, Université de Montréal, Montreal, Canada.

Classifications MeSH