FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation-Reference-Cited by-同舟云学术

FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation

Published:2021-07-23 Issue:30 Volume:7 Page:
ISSN:2375-2548
Container-title:Science Advances
language:en
Short-container-title:Sci. Adv.

Author:

Birsa Nicol¹²^ORCID,Ule Agnieszka M.¹^ORCID,Garone Maria Giovanna³⁴^ORCID,Tsang Brian⁵⁶^ORCID,Mattedi Francesca¹⁷^ORCID,Chong P. Andrew⁵^ORCID,Humphrey Jack¹^ORCID,Jarvis Seth¹,Pisiren Melis¹^ORCID,Wilkins Oscar G.¹⁸^ORCID,Nosella Micheal L.⁵,Devoy Anny⁹^ORCID,Bodo Cristian¹,de la Fuente Rafaela Fernandez¹,Fisher Elizabeth M. C.¹^ORCID,Rosa Alessandro³⁴^ORCID,Viero Gabriella⁷^ORCID,Forman-Kay Julie D.⁵⁶^ORCID,Schiavo Giampietro¹²^ORCID,Fratta Pietro¹¹⁰^ORCID

Affiliation:

1. Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London WC1N 3BG, UK.

2. UK Dementia Research Institute, University College London, London WC1E 6BT, UK.

3. Department of Biology and Biotechnology Charles Darwin, Sapienza University of Rome, P.le A. Moro 5, 00185 Rome, Italy.

4. Center for Life Nano Science, Istituto Italiano di Tecnologia, Viale Regina Elena 291, 00161 Rome, Italy.

5. Program in Molecular Medicine, The Hospital for Sick Children, Toronto, ON M5G 0A4, Canada.

6. Department of Biochemistry, University of Toronto, Toronto, ON M5S 1A8, Canada.

7. Institute of Biophysics, CNR, Trento, Italy.

8. The Francis Crick Institute, London NW1 1AT, UK.

9. Maurice Wohl Clinical Neuroscience Institute, King’s College London, London SE5 9RT, UK.

10. MRC Centre for Neuromuscular Disease, Queen Square, London WC1N 3BG, UK.

Abstract

Cytoplasmic mislocalization of FUS-ALS mutants determines aberrant FMRP condensates and protein synthesis repression.

Funder

Medical Research Council

Motor Neurone Disease Association

Rosetrees Trust

UCLH Biomedical Research Centre

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

Reference73 articles.

1. TDP-43 and FUSen routefrom the nucleus to the cytoplasm

2. Cytoplasmic functions of TDP-43 and FUS and their role in ALS

3. ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

4. Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss

5. Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

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