Translation of dipeptide repeat proteins from the C9ORF72 expanded repeat is associated with cellular stress

Translation of dipeptide repeat proteins from the C9ORF72 expanded repeat is associated with cellular stress

Expansion of a hexanucleotide repeat (HRE), GGGGCC, in the C9ORF72 gene is recognized as the most common cause of familial amyotrophic lateral sclerosis (FALS), frontotemporal dementia (FTD) and ALS-FTD, as well as 5–10% of sporadic ALS. Despite the location of the HRE in the non-coding region (with...

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Bibliographic Details
Main Authors: Yoshifumi Sonobe, Ghanashyam Ghadge, Katsuhisa Masaki, Ataman Sendoel, Elaine Fuchs, Raymond P. Roos
Format: Article
Language:English
Published: Elsevier 2018-08-01
Series:Neurobiology of Disease
Subjects:
C9ORF72
Dipeptide protein repeats (DPRs)
Hexanucleotide repeat expansions (HREs)
Repeat associated non-AUG (RAN) translation
Unconventional translation
Internal ribosome entry site (IRES)
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996118301475

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http://www.sciencedirect.com/science/article/pii/S0969996118301475

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