The carboxyl termini of RAN translated GGGGCC nucleotide repeat expansions modulate toxicity in models of ALS/FTD

The carboxyl termini of RAN translated GGGGCC nucleotide repeat expansions modulate toxicity in models of ALS/FTD

Abstract An intronic hexanucleotide repeat expansion in C9ORF72 causes familial and sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). This repeat is thought to elicit toxicity through RNA mediated protein sequestration and repeat-associated non-AUG (RAN) translation of...

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Bibliographic Details
Main Authors: Fang He, Brittany N. Flores, Amy Krans, Michelle Frazer, Sam Natla, Sarjina Niraula, Olamide Adefioye, Sami J. Barmada, Peter K. Todd
Format: Article
Language:English
Published: BMC 2020-08-01
Series:Acta Neuropathologica Communications
Subjects:
C9ORF72
Amyotrophic lateral sclerosis
Dipeptide repeat proteins (DPRs)
Repeat-associated non-AUG translation (RAN)
Carboxyl terminus
Drosophila
Online Access:http://link.springer.com/article/10.1186/s40478-020-01002-8

Internet

http://link.springer.com/article/10.1186/s40478-020-01002-8

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