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RNA‐binding proteins implicated in neurodegenerative diseases
- Source :
- WIREs RNA. 8
- Publication Year :
- 2016
- Publisher :
- Wiley, 2016.
-
Abstract
- Gene expression is regulated at many levels, including after generation of the primary RNA transcript from DNA but before translation into protein. Such post-translational gene regulation occurs via the action of a multitude of RNA binding proteins and include varied actions from splicing to regulation of association with the translational machinery. Primary evidence that such processes might contribute to disease mechanisms in neurodegenerative disorders comes from the observation of mutations in RNA binding proteins, particularly in diseases in the amyotrophic lateral sclerosis-frontotemporal dementia spectrum and in some forms of ataxia and tremor. The bulk of evidence from recent surveys of the types of RNA species that are affected in these disorders suggests a global deregulation of control rather than a very small number of RNA species, although why some groups of neurons are sensitive to these changes is not well understood. Overall, these data suggest that neurodegeneration can be initiated by mutations in RNA binding proteins and, as a corollary, that neurons are particularly sensitive to loss of control of gene expression at the post-transcriptional level. Such observations have implications not only for understanding the nature of neurodegenerative disorders but also how we might intervene therapeutically in these diseases. WIREs RNA 2017, 8:e1397. doi: 10.1002/wrna.1397 For further resources related to this article, please visit the WIREs website.
- Subjects :
- 0301 basic medicine
RNA-binding protein
Biology
Biochemistry
Article
03 medical and health sciences
chemistry.chemical_compound
0302 clinical medicine
Gene expression
medicine
Animals
Humans
Molecular Biology
Genetics
Regulation of gene expression
Neurodegeneration
RNA-Binding Proteins
RNA
Neurodegenerative Diseases
medicine.disease
Primary RNA Transcript
030104 developmental biology
chemistry
RNA splicing
030217 neurology & neurosurgery
DNA
Subjects
Details
- ISSN :
- 17577012 and 17577004
- Volume :
- 8
- Database :
- OpenAIRE
- Journal :
- WIREs RNA
- Accession number :
- edsair.doi.dedup.....1eab1180659eed2c97ac54114df0cc03