Cargando…
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: | 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: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2019
|
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 |
Ejemplares similares
-
Loss of Tmem106b is unable to ameliorate frontotemporal dementia-like phenotypes in an AAV mouse model of C9ORF72-repeat induced toxicity
por: Nicholson, Alexandra M., et al.
Publicado: (2018) -
Rodent Models of TDP-43 Proteinopathy: Investigating the Mechanisms of TDP-43-Mediated Neurodegeneration
por: Gendron, Tania F., et al.
Publicado: (2011) -
Mechanisms and models of TDP-43 proteinopathies
por: Petrucelli, Leonard
Publicado: (2012) -
Microglial lysosome dysfunction contributes to white matter pathology and TDP-43 proteinopathy in GRN-associated FTD
por: Wu, Yanwei, et al.
Publicado: (2021) -
Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS
por: Gendron, Tania F., et al.
Publicado: (2013)