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RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis

Gene transcription produces a wide variety of ribonucleic acid (RNA) species in eukaryotes. Individual types of RNA, such as messenger, structural and regulatory RNA, are known to play distinct roles in the cell. Recently, researchers have identified a large number of RNA-mediated toxicity pathways...

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Autor principal: Chan, Ho Yin Edwin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4271607/
https://www.ncbi.nlm.nih.gov/pubmed/25565965
http://dx.doi.org/10.3389/fncel.2014.00431
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author Chan, Ho Yin Edwin
author_facet Chan, Ho Yin Edwin
author_sort Chan, Ho Yin Edwin
collection PubMed
description Gene transcription produces a wide variety of ribonucleic acid (RNA) species in eukaryotes. Individual types of RNA, such as messenger, structural and regulatory RNA, are known to play distinct roles in the cell. Recently, researchers have identified a large number of RNA-mediated toxicity pathways that play significant pathogenic roles in numerous human disorders. In this article, we describe various common RNA toxicity pathways, namely epigenetic gene silencing, nucleolar stress, nucleocytoplasmic transport, bi-directional gene transcription, repeat-associated non-ATG translation, RNA foci formation and cellular protein sequestration. We emphasize RNA toxicity mechanisms that involve nucleotide repeat expansion, such as those related to polyglutamine (polyQ) disorders and frontotemporal lobar degeneration-amyotrophic lateral sclerosis.
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spelling pubmed-42716072015-01-06 RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis Chan, Ho Yin Edwin Front Cell Neurosci Neuroscience Gene transcription produces a wide variety of ribonucleic acid (RNA) species in eukaryotes. Individual types of RNA, such as messenger, structural and regulatory RNA, are known to play distinct roles in the cell. Recently, researchers have identified a large number of RNA-mediated toxicity pathways that play significant pathogenic roles in numerous human disorders. In this article, we describe various common RNA toxicity pathways, namely epigenetic gene silencing, nucleolar stress, nucleocytoplasmic transport, bi-directional gene transcription, repeat-associated non-ATG translation, RNA foci formation and cellular protein sequestration. We emphasize RNA toxicity mechanisms that involve nucleotide repeat expansion, such as those related to polyglutamine (polyQ) disorders and frontotemporal lobar degeneration-amyotrophic lateral sclerosis. Frontiers Media S.A. 2014-12-19 /pmc/articles/PMC4271607/ /pubmed/25565965 http://dx.doi.org/10.3389/fncel.2014.00431 Text en Copyright © 2014 Chan. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution and reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Chan, Ho Yin Edwin
RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis
title RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis
title_full RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis
title_fullStr RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis
title_full_unstemmed RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis
title_short RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis
title_sort rna-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4271607/
https://www.ncbi.nlm.nih.gov/pubmed/25565965
http://dx.doi.org/10.3389/fncel.2014.00431
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