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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...

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Detalles Bibliográficos
Autores principales: Lee, Paul Jongseo, Yang, Suzhou, Sun, Yu, Guo, Junjie U
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
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.
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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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