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Measuring RAN Peptide Toxicity in C. elegans
Published on: April 30, 2020
Pathogenic RNAs in microsatellite expansion disease
Jihae Shin1, Konstantinos Charizanis, Maurice S Swanson
1Department of Molecular Genetics and Microbiology and the Genetics Institute, University of Florida, College of Medicine, 1376 Mowry Road, Gainesville, FL, USA.
Neuroscience Letters
|August 4, 2009
Summary
Unstable microsatellite expansion causes neurological disorders. Toxic RNA transcripts are implicated, but both RNA and protein may contribute to disease, especially as the genome
Area of Science:
- Genetics
- Neuroscience
- Molecular Biology
Background:
- Microsatellite expansion diseases are inherited neurological and neuromuscular disorders.
- Pathogenesis is often linked to toxic RNA transcripts from expanded repeats in non-coding gene regions.
Purpose of the Study:
- To review the RNA-mediated toxicity model in microsatellite expansion diseases.
- To explore the combined roles of RNA and protein in disease pathogenesis.
Main Methods:
- Literature review of studies on microsatellite expansion disorders.
- Analysis of the impact of pathogenic RNAs on nervous system function.
- Exploration of recent genomic and transcriptomic findings.
Main Results:
- Evidence supports RNA toxicity as a key mechanism in several neurologic disorders.
- The distinction between coding and non-coding regions is becoming less clear.
- Combined RNA and protein gain-of-function may underlie some diseases.
Conclusions:
- Pathogenic RNAs can negatively impact nervous system functions.
- Microsatellite expansion diseases may involve complex pathogenic mechanisms.
- Further research is needed to elucidate the interplay of RNA and protein in these disorders.
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