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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Bibliographic Details
Published in:Frontiers in cellular neuroscience 2014-12, Vol.8, p.431-431
Main Author: Chan, Ho Yin Edwin
Format: Article
Language:English
Subjects:
Amyotrophic lateral sclerosis
Ataxia
Brain research
C9orf72
Degeneration
Deoxyribonucleic acid
Disease
DNA
Drosophila models
Frontotemporal dementia
Gene silencing
Genomes
Insects
Investigations
Life sciences
Mutation
Nematodes
Neurodegeneration
Neuroscience
Neurotoxicity
Nucleoli
nucleolin
Pathogenesis
polyglutamine disease
Polyglutamine diseases
Proteins
repeat expansion-associated non-ATG translation
Ribonucleic acid
RNA
RNA polymerase
RNA transport
Roles
Toxicity
Transcription
Trinucleotide repeat diseases
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Summary: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.
ISSN:1662-5102
1662-5102
DOI:10.3389/fncel.2014.00431