ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

Aarti Sharma; Alexander K. Lyashchenko; Lei Lu; Sara Ebrahimi Nasrabady; Margot Elmaleh; Monica Mendelsohn; Adriana Nemes; Juan Carlos Tapia; George Z. Mentis; Neil A. Shneider

doi:10.1038/ncomms10465

Nature Communications (Feb 2016)

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

Aarti Sharma,
Alexander K. Lyashchenko,
Lei Lu,
Sara Ebrahimi Nasrabady,
Margot Elmaleh,
Monica Mendelsohn,
Adriana Nemes,
Juan Carlos Tapia,
George Z. Mentis,
Neil A. Shneider

Affiliations

Aarti Sharma: Department of Neurology, Center for Motor Neuron Biology and Disease, Columbia University, 630 W 168th Street, P&S Building, Room 5-423
Alexander K. Lyashchenko: Department of Neurology, Center for Motor Neuron Biology and Disease, Columbia University, 630 W 168th Street, P&S Building, Room 5-423
Lei Lu: Department of Neurology, Center for Motor Neuron Biology and Disease, Columbia University, 630 W 168th Street, P&S Building, Room 5-423
Sara Ebrahimi Nasrabady: Department of Pathology and Cell Biology, Center for Motor Neuron Biology and Disease, Columbia University
Margot Elmaleh: Department of Neurology, Center for Motor Neuron Biology and Disease, Columbia University, 630 W 168th Street, P&S Building, Room 5-423
Monica Mendelsohn: Department of Pathology and Cell Biology, Center for Motor Neuron Biology and Disease, Columbia University
Adriana Nemes: Department of Neuroscience, Howard Hughes Medical Institute, Columbia University
Juan Carlos Tapia: Department of Neuroscience, Columbia University
George Z. Mentis: Department of Pathology and Cell Biology, Center for Motor Neuron Biology and Disease, Columbia University
Neil A. Shneider: Department of Neurology, Center for Motor Neuron Biology and Disease, Columbia University, 630 W 168th Street, P&S Building, Room 5-423

DOI: https://doi.org/10.1038/ncomms10465
Journal volume & issue: Vol. 7, no. 1
pp. 1 – 14

Abstract

Read online

The mechanism by which FUS mutations cause familial ALS remains unclear. Here, the authors use mouse transgenic models to show that a toxic gain-of-function underlies motor neuron degeneration, and that the toxicity of mutant FUS does not depend on a loss or excess of FUS activity.

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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