Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD

Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD

Mechanisms of transcription of expanded G4C2 repeats in C9ORF72, associated with ALS/FTD, are not fully understood. Here authors use both Drosophila and C9ORF72 iPSC-derived neurons and identify AFF2/FMR2 as a regulator of poly(GR) toxicity by regulating expression of the expanded G4C2 repeats.

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Autores principales: Yeliz Yuva-Aydemir, Sandra Almeida, Gopinath Krishnan, Tania F. Gendron, Fen-Biao Gao
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2019
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Acceso en línea:https://doaj.org/article/785bcaac78ea4e30bb76e6c0c40d7221
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spelling oai:doaj.org-article:785bcaac78ea4e30bb76e6c0c40d72212021-12-02T14:38:38ZTranscription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD10.1038/s41467-019-13477-82041-1723https://doaj.org/article/785bcaac78ea4e30bb76e6c0c40d72212019-11-01T00:00:00Zhttps://doi.org/10.1038/s41467-019-13477-8https://doaj.org/toc/2041-1723Mechanisms of transcription of expanded G4C2 repeats in C9ORF72, associated with ALS/FTD, are not fully understood. Here authors use both Drosophila and C9ORF72 iPSC-derived neurons and identify AFF2/FMR2 as a regulator of poly(GR) toxicity by regulating expression of the expanded G4C2 repeats.Yeliz Yuva-AydemirSandra AlmeidaGopinath KrishnanTania F. GendronFen-Biao GaoNature PortfolioarticleScienceQENNature Communications, Vol 10, Iss 1, Pp 1-14 (2019)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Yeliz Yuva-Aydemir
Sandra Almeida
Gopinath Krishnan
Tania F. Gendron
Fen-Biao Gao
Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD
description Mechanisms of transcription of expanded G4C2 repeats in C9ORF72, associated with ALS/FTD, are not fully understood. Here authors use both Drosophila and C9ORF72 iPSC-derived neurons and identify AFF2/FMR2 as a regulator of poly(GR) toxicity by regulating expression of the expanded G4C2 repeats.
format article
author Yeliz Yuva-Aydemir
Sandra Almeida
Gopinath Krishnan
Tania F. Gendron
Fen-Biao Gao
author_facet Yeliz Yuva-Aydemir
Sandra Almeida
Gopinath Krishnan
Tania F. Gendron
Fen-Biao Gao
author_sort Yeliz Yuva-Aydemir
title Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD
title_short Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD
title_full Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD
title_fullStr Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD
title_full_unstemmed Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD
title_sort transcription elongation factor aff2/fmr2 regulates expression of expanded ggggcc repeat-containing c9orf72 allele in als/ftd
publisher Nature Portfolio
publishDate 2019
url https://doaj.org/article/785bcaac78ea4e30bb76e6c0c40d7221
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AT gopinathkrishnan transcriptionelongationfactoraff2fmr2regulatesexpressionofexpandedggggccrepeatcontainingc9orf72alleleinalsftd
AT taniafgendron transcriptionelongationfactoraff2fmr2regulatesexpressionofexpandedggggccrepeatcontainingc9orf72alleleinalsftd
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