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Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
Published on: January 8, 2015
Comparative prion disease gene expression profiling using the prion disease mimetic, cuprizone
Laura R Moody1, Allen J Herbst, Han Sang Yoo
1Program in Cellular and Molecular Biology and Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin, Madison, WI, USA.
Prion
|June 19, 2009
Summary
Identifying novel gene expression changes in prion disease is crucial for finding biomarkers and therapies. This study uses cuprizone to control for cell changes, revealing 17 new transcripts specific to prion infection.
Area of Science:
- Neuroscience
- Molecular Biology
- Genetics
Background:
- Prion diseases cause neuropathology with complex gene expression changes.
- Identifying specific prion-induced gene alterations is challenging due to confounding cell population shifts.
- Cuprizone, a neurotoxicant, mimics prion disease-related cellular changes, offering a potential control model.
Purpose of the Study:
- To identify novel genes and transcripts specifically altered by prion infection.
- To utilize cuprizone as a control to differentiate prion-specific gene expression changes from general neuropathological responses.
- To discover potential biomarkers and therapeutic targets for prion diseases.
Main Methods:
- Gene expression profiling of mouse brains infected with Rocky Mountain Laboratory (RML) scrapie agent using Affymetrix gene arrays.
- Comparative analysis with gene expression profiles from cuprizone-treated mice to control for non-specific cellular changes.
- Identification of transcripts showing increased expression during prion infection but unaffected by cuprizone treatment.
Main Results:
- Prion infection altered the expression of 164 genes, with 83 previously undescribed.
- Cuprizone treatment induced 307 differentially regulated transcript changes, including spongiosis and glial fibrillary acidic protein (Gfap) activation.
- Comparative analysis identified 17 transcripts specifically upregulated during prion infection and unaffected by cuprizone.
Conclusions:
- Cuprizone serves as an effective control for cell population changes in prion disease research.
- This approach successfully identified novel transcripts associated with prion neuropathology.
- The identified transcripts represent potential biomarkers and therapeutic targets for prion diseases.
