Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy

Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy

Abstract Background A G4C2 hexanucleotide repeat expansion in the noncoding region of C9orf72 is the major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis (c9FTD/ALS). Putative disease mechanisms underlying c9FTD/ALS include toxicity from sense G4C2 and antisense G2C4 repe...

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Bibliographic Details
Main Authors: Jeannie Chew, Casey Cook, Tania F. Gendron, Karen Jansen-West, Giulia del Rosso, Lillian M. Daughrity, Monica Castanedes-Casey, Aishe Kurti, Jeannette N. Stankowski, Matthew D. Disney, Jeffrey D. Rothstein, Dennis W. Dickson, John D. Fryer, Yong-Jie Zhang, Leonard Petrucelli
Format: Article
Language:English
Published: BMC 2019-02-01
Series:Molecular Neurodegeneration
Subjects:
C9orf72
Stress granules
Frontotemporal dementia
Amyotrophic lateral sclerosis
TDP-43
Neurodegeneration
Online Access:http://link.springer.com/article/10.1186/s13024-019-0310-z

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