CUG initiation and frameshifting enable production of dipeptide repeat proteins from ALS/FTD C9ORF72 transcripts

Ricardos Tabet; Laure Schaeffer; Fernande Freyermuth; Melanie Jambeau; Michael Workman; Chao-Zong Lee; Chun-Chia Lin; Jie Jiang; Karen Jansen-West; Hussein Abou-Hamdan; Laurent Désaubry; Tania Gendron; Leonard Petrucelli; Franck Martin; Clotilde Lagier-Tourenne

doi:10.1038/s41467-017-02643-5

Nature Communications (Jan 2018)

CUG initiation and frameshifting enable production of dipeptide repeat proteins from ALS/FTD C9ORF72 transcripts

Ricardos Tabet,
Laure Schaeffer,
Fernande Freyermuth,
Melanie Jambeau,
Michael Workman,
Chao-Zong Lee,
Chun-Chia Lin,
Jie Jiang,
Karen Jansen-West,
Hussein Abou-Hamdan,
Laurent Désaubry,
Tania Gendron,
Leonard Petrucelli,
Franck Martin,
Clotilde Lagier-Tourenne

Affiliations

Ricardos Tabet: Department of Neurology, MassGeneral Institute for Neurodegenerative Disease (MIND), Massachusetts General Hospital and Harvard Medical School
Laure Schaeffer: Architecture et Réactivité de l’ARN, UPR 9002, Université de Strasbourg, CNRS
Fernande Freyermuth: Department of Neurology, MassGeneral Institute for Neurodegenerative Disease (MIND), Massachusetts General Hospital and Harvard Medical School
Melanie Jambeau: Department of Neurology, MassGeneral Institute for Neurodegenerative Disease (MIND), Massachusetts General Hospital and Harvard Medical School
Michael Workman: Department of Neurology, MassGeneral Institute for Neurodegenerative Disease (MIND), Massachusetts General Hospital and Harvard Medical School
Chao-Zong Lee: Department of Neurology, MassGeneral Institute for Neurodegenerative Disease (MIND), Massachusetts General Hospital and Harvard Medical School
Chun-Chia Lin: Department of Neurology, MassGeneral Institute for Neurodegenerative Disease (MIND), Massachusetts General Hospital and Harvard Medical School
Jie Jiang: Ludwig Institute for Cancer Research, University of California San Diego
Karen Jansen-West: Department of Neuroscience, Mayo Clinic
Hussein Abou-Hamdan: Therapeutic Innovation Laboratory (UMR 7200), Faculty of Pharmacy, CNRS/University of Strasbourg
Laurent Désaubry: Therapeutic Innovation Laboratory (UMR 7200), Faculty of Pharmacy, CNRS/University of Strasbourg
Tania Gendron: Department of Neuroscience, Mayo Clinic
Leonard Petrucelli: Department of Neuroscience, Mayo Clinic
Franck Martin: Architecture et Réactivité de l’ARN, UPR 9002, Université de Strasbourg, CNRS
Clotilde Lagier-Tourenne: Department of Neurology, MassGeneral Institute for Neurodegenerative Disease (MIND), Massachusetts General Hospital and Harvard Medical School

DOI: https://doi.org/10.1038/s41467-017-02643-5
Journal volume & issue: Vol. 9, no. 1
pp. 1 – 14

Abstract

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Repeat-associated non-AUG (RAN) translation contributes to the pathogenic mechanism of several microsatellite expansion diseases. Here the authors delineate the different steps involved in recruiting the ribosome to initiate G4C2 RAN translation to produce poly-Glycine Alanine, poly-Glycine Proline, and poly-Glycine Arginine 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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