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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Bibliographic Details
Main Authors: Yeliz Yuva-Aydemir, Sandra Almeida, Gopinath Krishnan, Tania F. Gendron, Fen-Biao Gao
Format: article
Language:EN
Published: Nature Portfolio 2019
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Online Access:https://doaj.org/article/785bcaac78ea4e30bb76e6c0c40d7221
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Summary: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.