Reference : Elongator subunit 3 (ELP3) modifies ALS through tRNA modification
Scientific journals : Article
Human health sciences : Neurology
http://hdl.handle.net/2268/243284
Elongator subunit 3 (ELP3) modifies ALS through tRNA modification
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Bento-Abreu, A. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Jager, G. [Department of Molecular Biology, Umeå University, Umeå, 901 87, Sweden]
Swinnen, B. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium, Department of Neurology, University Hospitals Leuven, Leuven, B-3000, Belgium]
Rué, L. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Hendrickx, Sébastien [Université de Liège - ULiège > > Lepur(Centre rech.sur la Ville,le Territ.et le Milieu rural) >]
Jones, A. [Department of Clinical Neuroscience, Institute of Psychiatry, King's College London, London, SE5 8AF, United Kingdom]
Staats, K. A. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Taes, I. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Eykens, C. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Nonneman, A. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Nuyts, R. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Timmers, M. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Silva, L. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Chariot, Alain mailto [Université de Liège - ULiège > Département de pharmacie > Chimie médicale >]
Nguyen, Laurent mailto [Université de Liège - ULiège > > Neurosciences-Molecular Regulation of Neurogenesis >]
Ravits, J. [Department of Neurosciences, ALS Translational Research, University of California, San Diego, La Jolla, CA, United States]
Lemmens, R. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium, Department of Neurology, University Hospitals Leuven, Leuven, B-3000, Belgium]
Cabooter, D. [Department of Pharmaceutical and Pharmacological Sciences, Pharmaceutical Analysis, Leuven, B-3000, Belgium]
Van Den Bosch, L. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium]
Van Damme, P. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Laboratory of Neurobiology, VIB-Center for Brain and Disease Research, Leuven, B-3000, Belgium, Department of Neurology, University Hospitals Leuven, Leuven, B-3000, Belgium]
Al-Chalabi, A. [Department of Clinical Neuroscience, Institute of Psychiatry, King's College London, London, SE5 8AF, United Kingdom]
Bystrom, A. [Department of Molecular Biology, Umeå University, Umeå, 901 87, Sweden]
Robberecht, W. [Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease (LIND), KU Leuven-University of Leuven, Leuven, B-3000, Belgium, Department of Neurology, University Hospitals Leuven, Leuven, B-3000, Belgium]
2018
Human Molecular Genetics
Oxford University Press
27
7
1276-1289
Yes (verified by ORBi)
International
0964-6906
1460-2083
[en] ELP3 protein, zebrafish ; Article ; RNA processing ; Amyotrophic Lateral Sclerosis ; Animals ; C9orf72 Protein ; Histone Acetyltransferases ; Motor Cortex ; Nerve Tissue Proteins ; RNA Processing, Post-Transcriptional ; RNA, Transfer ; Superoxide Dismutase-1 ; Zebrafish
[en] Amyotrophic lateral sclerosis (ALS) is a fatal degenerative motor neuron disorder of which the progression is influenced by several disease-modifying factors. Here, we investigated ELP3, a subunit of the elongator complex that modifies tRNA wobble uridines, as one of such ALS disease modifiers. ELP3 attenuated the axonopathy of a mutant SOD1, as well as of a mutant C9orf72 ALS zebrafish model. Furthermore, the expression of ELP3 in the SOD1G93A mouse extended the survival and attenuated the denervation in this model. Depletion of ELP3 in vitro reduced the modified tRNA wobble uridine mcm5s2U and increased abundance of insoluble mutant SOD1, which was reverted by exogenous ELP3 expression. Interestingly, the expression of ELP3 in the motor cortex of ALS patients was reduced and correlated with mcm5s2U levels. Our results demonstrate that ELP3 is a modifier of ALS and suggest a link between tRNA modification and neurodegeneration. © The Author(s) 2018. Published by Oxford University Press. All rights reserved.
Researchers ; Professionals ; Students ; General public
http://hdl.handle.net/2268/243284
10.1093/hmg/ddy043

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