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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)...
Autores principales: | , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6377782/ https://www.ncbi.nlm.nih.gov/pubmed/30767771 http://dx.doi.org/10.1186/s13024-019-0310-z |