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Updated: Jul 25, 2026
Protein Misfolding Cyclic Amplification of Prions
Published on: November 7, 2012
Dimethyl sulfoxide delays PrP sc accumulation and disease symptoms in prion-infected hamsters
Gideon M Shaked1, Roni Engelstein, Inbal Avraham
1Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital, 91120, Jerusalem, Israel.
Abstract:
PrP(Sc), an aberrantly folded protein, is the only identified component of the prion, an agent causing fatal neurodegenerative diseases such as scrapie and bovine spongiform encephalopathy. Dimethyl sulfoxide (DMSO) has been shown to reduce the accumulation of PrP(Sc) in scrapie-infected (ScN2a) cells, and to inhibit its aggregation in vitro. In humans, DMSO was used successfully in the treatment of various peripheral amyloidotic diseases. Here we show that administration of DMSO to scrapie-infected hamsters significantly prolonged disease incubation time, as well as delayed the accumulation of PrP(Sc) in Syrian hamster brains. Interestingly, administration of DMSO to scrapie sick hamsters resulted in increased clearance of protease-resistant PrP in their urine. We conclude that although DMSO by itself may not be sufficient to cure prion diseases, it may be considered as a component in a 'cocktail' drug approach for these disorders. Also, urine PrP testing should be considered for the assessment of treatment efficacy.
Insights
Dimethyl sulfoxide (DMSO) prolonged prion disease incubation in hamsters and reduced abnormal prion protein (PrPSc) accumulation. Urine testing for PrPSc may help assess treatment effectiveness for these neurodegenerative diseases.
Area of Science:
- Neuroscience
- Biochemistry
- Pharmacology
Background:
- Prion diseases, such as scrapie and bovine spongiform encephalopathy, are fatal neurodegenerative disorders.
- The disease is caused by an abnormal prion protein (PrPSc).
- Dimethyl sulfoxide (DMSO) has shown potential in reducing PrPSc accumulation and aggregation in vitro and in treating human amyloid diseases.
Purpose of the Study:
- To investigate the efficacy of DMSO in treating prion diseases in vivo.
- To determine DMSO's effect on disease progression and PrPSc levels in scrapie-infected hamsters.
Main Methods:
- Administration of DMSO to scrapie-infected hamsters.
- Monitoring of disease incubation time.
- Quantification of PrPSc accumulation in hamster brains.
- Analysis of protease-resistant PrP levels in urine.
Main Results:
- DMSO administration significantly prolonged the incubation period of scrapie in hamsters.
- DMSO treatment delayed the accumulation of PrPSc in the brains of infected hamsters.
- Scrapie-infected hamsters treated with DMSO showed increased clearance of protease-resistant PrP in their urine.
Conclusions:
- DMSO may be a potential component in combination therapies for prion diseases.
- Urine PrP testing could serve as a valuable biomarker for evaluating treatment efficacy in prion diseases.
