دورية أكاديمية
Elongator subunit 3 (ELP3) modifies ALS through tRNA modification
| العنوان: | Elongator subunit 3 (ELP3) modifies ALS through tRNA modification |
|---|---|
| المؤلفون: | Bento-Abreu, A., Jager, G., Swinnen, B., Rué, L., Hendrickx, Sébastien, Jones, A., Staats, K. A., Taes, I., Eykens, C., Nonneman, A., Nuyts, R., Timmers, M., Silva, L., Chariot, Alain, Nguyen, Laurent, Ravits, J., Lemmens, R., Cabooter, D., Van Den Bosch, L., Van Damme, P., Al-Chalabi, A., Bystrom, A., Robberecht, W. |
| المصدر: | Human Molecular Genetics, 27 (7), 1276-1289 (2018) |
| بيانات النشر: | Oxford University Press |
| سنة النشر: | 2018 |
| المجموعة: | University of Liège: ORBi (Open Repository and Bibliography) |
| مصطلحات موضوعية: | ELP3 protein, zebrafish, Article, RNA processing, Amyotrophic Lateral Sclerosis, Animals, C9orf72 Protein, Histone Acetyltransferases, Motor Cortex, Nerve Tissue Proteins, Post-Transcriptional, RNA, Transfer, Superoxide Dismutase-1, Human health sciences, Neurology, Sciences de la santé humaine, Neurologie |
| الوصف: | peer reviewed ; 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. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | unknown |
| تدمد: | 0964-6906 1460-2083 |
| العلاقة: | urn:issn:0964-6906; urn:issn:1460-2083; https://orbi.uliege.be/handle/2268/243284Test; info:hdl:2268/243284; https://orbi.uliege.be/bitstream/2268/243284/1/HMG%20%282018%29%20Bento_Abreu.pdfTest; scopus-id:2-s2.0-85044872158; info:pmid:29415125 |
| DOI: | 10.1093/hmg/ddy043 |
| الإتاحة: | https://doi.org/10.1093/hmg/ddy043Test https://orbi.uliege.be/handle/2268/243284Test https://orbi.uliege.be/bitstream/2268/243284/1/HMG%20%282018%29%20Bento_Abreu.pdfTest |
| حقوق: | open access ; http://purl.org/coar/access_right/c_abf2Test ; info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.C640E4DD |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 09646906 14602083 |
|---|---|
| DOI: | 10.1093/hmg/ddy043 |