Protein disulphide isomerase (PDI) is protective against amyotrophic lateral sclerosis (ALS)-related mutant Fused in Sarcoma (FUS) in in vitro models

Abstract Mutations in Fused in Sarcoma (FUS) are present in familial and sporadic cases of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). FUS is localised in the nucleus where it has important functions in DNA repair. However, in ALS/FTD, mutant FUS mislocalises from the nucl...

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Autores principales: S. Parakh, E. R. Perri, M. Vidal, J. Sultana, S. Shadfar, P. Mehta, A. Konopka, C. J. Thomas, D. M. Spencer, J. D. Atkin
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2021
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Acceso en línea:https://doaj.org/article/bb745be584d64c03bede47b9c4477073
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