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Regulation of nonsense-mediated mRNA decay in neural development and disease
Eukaryotes have evolved a variety of mRNA surveillance mechanisms to detect and degrade aberrant mRNAs with potential deleterious outcomes. Among them, nonsense-mediated mRNA decay (NMD) functions not only as a quality control mechanism targeting aberrant mRNAs containing a premature termination cod...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8339359/ https://www.ncbi.nlm.nih.gov/pubmed/33783512 http://dx.doi.org/10.1093/jmcb/mjab022 |
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author | Lee, Paul Jongseo Yang, Suzhou Sun, Yu Guo, Junjie U |
author_facet | Lee, Paul Jongseo Yang, Suzhou Sun, Yu Guo, Junjie U |
author_sort | Lee, Paul Jongseo |
collection | PubMed |
description | Eukaryotes have evolved a variety of mRNA surveillance mechanisms to detect and degrade aberrant mRNAs with potential deleterious outcomes. Among them, nonsense-mediated mRNA decay (NMD) functions not only as a quality control mechanism targeting aberrant mRNAs containing a premature termination codon but also as a posttranscriptional gene regulation mechanism targeting numerous physiological mRNAs. Despite its well-characterized molecular basis, the regulatory scope and biological functions of NMD at an organismal level are incompletely understood. In humans, mutations in genes encoding core NMD factors cause specific developmental and neurological syndromes, suggesting a critical role of NMD in the central nervous system. Here, we review the accumulating biochemical and genetic evidence on the developmental regulation and physiological functions of NMD as well as an emerging role of NMD dysregulation in neurodegenerative diseases. |
format | Online Article Text |
id | pubmed-8339359 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-83393592021-08-09 Regulation of nonsense-mediated mRNA decay in neural development and disease Lee, Paul Jongseo Yang, Suzhou Sun, Yu Guo, Junjie U J Mol Cell Biol Reviews Eukaryotes have evolved a variety of mRNA surveillance mechanisms to detect and degrade aberrant mRNAs with potential deleterious outcomes. Among them, nonsense-mediated mRNA decay (NMD) functions not only as a quality control mechanism targeting aberrant mRNAs containing a premature termination codon but also as a posttranscriptional gene regulation mechanism targeting numerous physiological mRNAs. Despite its well-characterized molecular basis, the regulatory scope and biological functions of NMD at an organismal level are incompletely understood. In humans, mutations in genes encoding core NMD factors cause specific developmental and neurological syndromes, suggesting a critical role of NMD in the central nervous system. Here, we review the accumulating biochemical and genetic evidence on the developmental regulation and physiological functions of NMD as well as an emerging role of NMD dysregulation in neurodegenerative diseases. Oxford University Press 2021-03-30 /pmc/articles/PMC8339359/ /pubmed/33783512 http://dx.doi.org/10.1093/jmcb/mjab022 Text en © The Author(s) (2021). Published by Oxford University Press on behalf of Journal of Molecular Cell Biology, CEMCS, CAS. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Reviews Lee, Paul Jongseo Yang, Suzhou Sun, Yu Guo, Junjie U Regulation of nonsense-mediated mRNA decay in neural development and disease |
title | Regulation of nonsense-mediated mRNA decay in neural development and disease |
title_full | Regulation of nonsense-mediated mRNA decay in neural development and disease |
title_fullStr | Regulation of nonsense-mediated mRNA decay in neural development and disease |
title_full_unstemmed | Regulation of nonsense-mediated mRNA decay in neural development and disease |
title_short | Regulation of nonsense-mediated mRNA decay in neural development and disease |
title_sort | regulation of nonsense-mediated mrna decay in neural development and disease |
topic | Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8339359/ https://www.ncbi.nlm.nih.gov/pubmed/33783512 http://dx.doi.org/10.1093/jmcb/mjab022 |
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