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...
Main Authors: | , , , , , , , , , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
BMC
2019-02-01
|
Series: | Molecular Neurodegeneration |
Subjects: | |
Online Access: | http://link.springer.com/article/10.1186/s13024-019-0310-z |