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

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

Abstract 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 pathogenes...

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Bibliographic Details
Main Authors: Daniel A. Mordes, Mercedes Prudencio, Lindsey D. Goodman, Joseph R. Klim, Rob Moccia, Francesco Limone, Olli Pietilainen, Kaitavjeet Chowdhary, Dennis W. Dickson, Rosa Rademakers, Nancy M. Bonini, Leonard Petrucelli, Kevin Eggan
Format: Article
Language:English
Published: BMC 2018-07-01
Series:Acta Neuropathologica Communications
Subjects:
Amyotrophic lateral sclerosis
C9ORF72 repeat expansion
Dipeptide repeat proteins
Drosophila
Frontotemporal dementia
Frontotemporal lobar degeneration
Online Access:http://link.springer.com/article/10.1186/s40478-018-0555-8

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http://link.springer.com/article/10.1186/s40478-018-0555-8

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