RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response

Katelyn M. Green; M. Rebecca Glineburg; Michael G. Kearse; Brittany N. Flores; Alexander E. Linsalata; Stephen J. Fedak; Aaron C. Goldstrohm; Sami J. Barmada; Peter K. Todd

doi:10.1038/s41467-017-02200-0

Nature Communications (Dec 2017)

RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response

Katelyn M. Green,
M. Rebecca Glineburg,
Michael G. Kearse,
Brittany N. Flores,
Alexander E. Linsalata,
Stephen J. Fedak,
Aaron C. Goldstrohm,
Sami J. Barmada,
Peter K. Todd

Affiliations

Katelyn M. Green: Department of Neurology, University of Michigan
M. Rebecca Glineburg: Department of Neurology, University of Michigan
Michael G. Kearse: Department of Neurology, University of Michigan
Brittany N. Flores: Department of Neurology, University of Michigan
Alexander E. Linsalata: Department of Neurology, University of Michigan
Stephen J. Fedak: Department of Neurology, University of Michigan
Aaron C. Goldstrohm: Department of Biochemistry, Molecular Biology, and Biophysics, University of Minnesota
Sami J. Barmada: Department of Neurology, University of Michigan
Peter K. Todd: Department of Neurology, University of Michigan

DOI: https://doi.org/10.1038/s41467-017-02200-0
Journal volume & issue: Vol. 8, no. 1
pp. 1 – 13

Abstract

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A nucleotide repeat expansion in C9orf72 is a common genetic cause of neurodegenerative disorders. Here, the authors provide insight into the molecular mechanism by which this repeat undergoes Repeat-Associated Non-AUG (RAN) translation, implicating the integrated stress response and eIF2α phosphorylation.

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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