Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation

Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation

Abstract Mutations in FUS, an RNA-binding protein involved in multiple steps of RNA metabolism, are associated with the most severe forms of amyotrophic lateral sclerosis (ALS). Accumulation of cytoplasmic FUS is likely to be a major culprit in the toxicity of FUS mutations. Thus, preventing cytopla...

Full description

Bibliographic Details
Main Authors: Inmaculada Sanjuan-Ruiz, Noé Govea-Perez, Melissa McAlonis-Downes, Stéphane Dieterle, Salim Megat, Sylvie Dirrig-Grosch, Gina Picchiarelli, Diana Piol, Qiang Zhu, Brian Myers, Chao-Zong Lee, Don W Cleveland, Clotilde Lagier-Tourenne, Sandrine Da Cruz, Luc Dupuis
Format: Article
Language:English
Published: BMC 2021-09-01
Series:Molecular Neurodegeneration
Subjects:
Amyotrophic lateral sclerosis
Fronto-temporal dementia
Mouse models
RNA-binding proteins
FUS
Autoregulation
Online Access:https://doi.org/10.1186/s13024-021-00477-w

Internet

https://doi.org/10.1186/s13024-021-00477-w

Similar Items