Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients

Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients

Hexanucleotide repeat expansion in the C9ORF72 gene results in production of dipeptide repeat (DPR) proteins that may disrupt pre-mRNA splicing in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) patients. At present, the mechanisms underlying this mis-splicing are not understoo...

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Bibliographic Details
Main Authors: Shanye Yin, Rodrigo Lopez-Gonzalez, Ryan C. Kunz, Jaya Gangopadhyay, Carl Borufka, Steven P. Gygi, Fen-Biao Gao, Robin Reed
Format: Article
Language:English
Published: Elsevier 2017-06-01
Series:Cell Reports
Subjects:
pre-mRNA splicing
U2 snRNP
ALS
FTD
C9ORF72
toxic polydipeptide repeats
DPRs
poly-GR
poly-PR
iPSC-derived motor neurons
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124717307131

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http://www.sciencedirect.com/science/article/pii/S2211124717307131

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