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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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Detalles Bibliográficos
Autores principales:	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:	Online Artículo Texto
Lenguaje:	English
Publicado:	BioMed Central 2018
Materias:	Research
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6031111/ https://www.ncbi.nlm.nih.gov/pubmed/29973287 http://dx.doi.org/10.1186/s40478-018-0555-8

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