Nature Communications (Dec 2020)
Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis
- Daniel Jutzi,
- Sébastien Campagne,
- Ralf Schmidt,
- Stefan Reber,
- Jonas Mechtersheimer,
- Foivos Gypas,
- Christoph Schweingruber,
- Martino Colombo,
- Christine von Schroetter,
- Fionna E. Loughlin,
- Anny Devoy,
- Eva Hedlund,
- Mihaela Zavolan,
- Frédéric H.-T. Allain,
- Marc-David Ruepp
Affiliations
- Daniel Jutzi
- United Kingdom Dementia Research Institute Centre, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute
- Sébastien Campagne
- Institute of Molecular Biology and Biophysics, Department of Biology, ETH Zürich
- Ralf Schmidt
- Computational and Systems Biology, Biozentrum, University of Basel
- Stefan Reber
- United Kingdom Dementia Research Institute Centre, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute
- Jonas Mechtersheimer
- United Kingdom Dementia Research Institute Centre, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute
- Foivos Gypas
- Computational and Systems Biology, Biozentrum, University of Basel
- Christoph Schweingruber
- Department of Neuroscience, Karolinska Institutet
- Martino Colombo
- Celgene Institute of Translational Research (CITRE)
- Christine von Schroetter
- Institute of Molecular Biology and Biophysics, Department of Biology, ETH Zürich
- Fionna E. Loughlin
- Institute of Molecular Biology and Biophysics, Department of Biology, ETH Zürich
- Anny Devoy
- United Kingdom Dementia Research Institute Centre, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute
- Eva Hedlund
- Department of Neuroscience, Karolinska Institutet
- Mihaela Zavolan
- Computational and Systems Biology, Biozentrum, University of Basel
- Frédéric H.-T. Allain
- Institute of Molecular Biology and Biophysics, Department of Biology, ETH Zürich
- Marc-David Ruepp
- United Kingdom Dementia Research Institute Centre, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute
- DOI
- https://doi.org/10.1038/s41467-020-20191-3
- Journal volume & issue
-
Vol. 11,
no. 1
pp. 1 – 14
Abstract
Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS). Here the authors characterize FUS-binding to U1 snRNP and show that ALS-associated FUS aberrantly contacts U1 snRNA which interferes with its biogenesis pathway.
