Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic.

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Title

Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic.

Content partner

The University of Auckland Library

Collection

ResearchSpace@Auckland

Description

The GGGGCC (G4C2) intronic repeat expansion within C9ORF72 is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Intranuclear neuronal RNA foci have been observed in ALS and FTD tissues, suggesting that G4C2 RNA may be toxic. Here, we demonstrate that the expression of 38× and 72× G4C2 repeats form intranuclear RNA foci that initiate apoptotic cell death in neuronal cell lines and zebrafish embryos. The foci colocalize with a subset of RNA ...

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Format

Research paper

Research format

Journal article

Date created

2013-12

Creator

Lee, YB / Chen, HJ / Peres, JN / Gomez-Deza, J / Attig, J / Stalekar, M / Troakes, C / Nishimura, AL / Scotter, Emma / Vance, C / Adachi, Y / Sardone, V / Miller, JW / Smith, BN / Gallo, JM / Ule, J / Hirth, F / Rogelj, B / Houart, C / Shaw, CE

URL

http://hdl.handle.net/2292/23498

Related subjects

Cell Line, Tumor / Animals / Zebrafish / Humans / Rats / Amyotrophic Lateral Sclerosis / Proteins / RNA-Binding Proteins / RNA, Messenger / Case-Control Studies / Apoptosis / RNA Splicing / Microsatellite Repeats / Protein Binding / Adult / Aged / Aged, 80 and over / Middle Aged / Female / Male / Frontotemporal Dementia

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Items in ResearchSpace are protected by copyright, with all rights reserved, unless otherwise indicated. Previously published items are made available in accordance with the copyright policy of the publisher. Details obtained from http://www.cell.com/cell-reports/authors http://www.sherpa.ac.uk/romeo/issn/2211-1247/

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• Items in ResearchSpace are protected by copyright, with all rights reserved, unless otherwise indicated. Previously published items are made available in accordance with the copyright policy of the publisher. Details obtained from http://www.cell.com/cell-reports/authors http://www.sherpa.ac.uk/romeo/issn/2211-1247/
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