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

Yeliz Yuva-Aydemir; Sandra Almeida; Gopinath Krishnan; Tania F. Gendron; Fen-Biao Gao

doi:10.1038/s41467-019-13477-8

Nature Communications (Nov 2019)

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

Yeliz Yuva-Aydemir,
Sandra Almeida,
Gopinath Krishnan,
Tania F. Gendron,
Fen-Biao Gao

Affiliations

Yeliz Yuva-Aydemir: Department of Neurology, University of Massachusetts Medical School
Sandra Almeida: Department of Neurology, University of Massachusetts Medical School
Gopinath Krishnan: Department of Neurology, University of Massachusetts Medical School
Tania F. Gendron: Department of Neuroscience, Mayo Clinic Florida
Fen-Biao Gao: Department of Neurology, University of Massachusetts Medical School

DOI: https://doi.org/10.1038/s41467-019-13477-8
Journal volume & issue: Vol. 10, no. 1
pp. 1 – 14

Abstract

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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.

Published in Nature Communications

ISSN: 2041-1723 (Online)
Publisher: Nature Portfolio
Country of publisher: United Kingdom
LCC subjects: Science
Website: https://www.nature.com/ncomms/

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