Inducible expression of human C9ORF72 36× G4C2 hexanucleotide repeats is sufficient to cause RAN translation and rapid muscular atrophy in mice

The hexanucleotide G4C2 repeat expansion in the first intron of the C9ORF72 gene accounts for the majority of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) cases. Numerous studies have indicated the toxicity of dipeptide repeats (DPRs), which are produced via repeat-associate...

Full description

Bibliographic Details
Main Authors: Fréderike W. Riemslagh, Esmay C. van der Toorn, Rob F. M. Verhagen, Alex Maas, Laurens W. J. Bosman, Renate K. Hukema, Rob Willemsen
Format: Article
Language:English
Published: The Company of Biologists 2021-02-01
Series:Disease Models & Mechanisms
Subjects:
Online Access:http://dmm.biologists.org/content/14/2/dmm044842