Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients

A hexanucleotide (GGGGCC) repeat expansion in C9ORF72 is the most common genetic contributor to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Reduced expression of the C9ORF72 gene product has been proposed as a potential contributor to disease pathogenesis. Addit...

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Detalhes bibliográficos
Publicado no:Acta Neuropathol Commun
Main Authors: Mordes, Daniel A., Prudencio, Mercedes, Goodman, Lindsey D., Klim, Joseph R., Moccia, Rob, Limone, Francesco, Pietilainen, Olli, Chowdhary, Kaitavjeet, Dickson, Dennis W., Rademakers, Rosa, Bonini, Nancy M., Petrucelli, Leonard, Eggan, Kevin
Formato: Artigo
Idioma:Inglês
Publicado em: BioMed Central 2018
Assuntos:
Research
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6031111/
https://ncbi.nlm.nih.gov/pubmed/29973287
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s40478-018-0555-8
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