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RNA-binding proteins implicated in neurodegenerative diseases.
1Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, MD, USA.
Wiley Interdisciplinary Reviews. RNA
|September 24, 2016
Summary
Mutations in RNA binding proteins can initiate neurodegenerative diseases by disrupting gene expression control. Neurons are especially vulnerable to these post-transcriptional gene regulation defects.
Area of Science:
- Molecular Biology
- Neuroscience
- Genetics
Background:
- Gene expression is regulated post-transcriptionally by RNA binding proteins.
- Dysregulation of these processes is implicated in neurodegenerative disorders.
Purpose of the Study:
- To explore the role of post-transcriptional gene regulation in neurodegeneration.
- To understand the link between RNA binding protein mutations and neuronal vulnerability.
Main Methods:
- Review of existing literature on RNA binding proteins and neurodegenerative diseases.
- Analysis of RNA species affected in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorders, ataxia, and tremor.
Main Results:
- Mutations in RNA binding proteins are linked to neurodegenerative diseases.
- Evidence suggests global deregulation of gene expression rather than specific RNA species.
- Neurons exhibit particular sensitivity to post-transcriptional gene control loss.
Conclusions:
- Neurodegeneration can be initiated by mutations affecting RNA binding proteins.
- Neurons are highly sensitive to disruptions in post-transcriptional gene regulation.
- Findings have implications for understanding and treating neurodegenerative diseases.
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