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

BACKGROUND: A G(4)C(2) 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 G(4)C(2) and antisense G(2)C(4)...

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Publicado en:Mol Neurodegener
Autores principales: Chew, Jeannie, Cook, Casey, Gendron, Tania F., Jansen-West, Karen, del Rosso, Giulia, Daughrity, Lillian M., Castanedes-Casey, Monica, Kurti, Aishe, Stankowski, Jeannette N., Disney, Matthew D., Rothstein, Jeffrey D., Dickson, Dennis W., Fryer, John D., Zhang, Yong-Jie, Petrucelli, Leonard
Formato: Artigo
Lenguaje:Inglês
Publicado: BioMed Central 2019
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Research Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC6377782/
https://ncbi.nlm.nih.gov/pubmed/30767771
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s13024-019-0310-z
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