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Unchanged survival rates of Shadoo knockout mice after infection with mouse-adapted scrapie
Sha Li1, Chuanjing Ju, Chao Han
1a Institute of Military Veterinary; Academy of Military Medical Science ; Changchun , China.
Abstract:
Previous studies have demonstrated that Shadoo (Sho), a GPI-linked glycoprotein encoded by the Sprn gene with a membrane localization similar to PrP(C), is reduced in the brains of rodents with terminal prion disease. To determine the functional significance of Sho in prion disease pathogenesis, Sho-deficient mice were generated by gene targeting. Sho knockout and control wild-type (WT) mice were infected with themouse-adapted scrapie strains 22L or RML. No significant differences in survival, the incubation period of prion disease or other disease features were observed between Sho mutant and WT mice. In this model of prion disease, Sho removal had no effect on disease pathogenesis.
Insights
Shadoo (Sho) protein levels decrease in prion disease. However, removing Sho in mice did not alter prion disease progression or survival, suggesting Sho is not critical for pathogenesis.
Area of Science:
- Neuroscience
- Prion Disease Research
- Molecular Biology
Background:
- Shadoo (Sho) is a GPI-linked glycoprotein with membrane localization similar to PrP(C).
- Sho levels are reduced in the brains of rodents with terminal prion disease.
- The functional significance of Sho in prion disease pathogenesis remains unclear.
Purpose of the Study:
- To investigate the functional role of Shadoo (Sho) in prion disease pathogenesis.
- To determine if Sho deficiency impacts disease progression and survival in a mouse model of prion disease.
Main Methods:
- Generation of Sho-deficient (knockout) mice using gene targeting.
- Infection of Sho knockout and wild-type (WT) control mice with mouse-adapted scrapie strains (22L or RML).
- Monitoring and comparison of disease features, including survival and incubation period, between knockout and WT groups.
Main Results:
- No significant differences in survival rates were observed between Sho-deficient and WT mice.
- The incubation period of prion disease did not differ significantly between the two groups.
- Sho removal had no discernible effect on other observed prion disease features.
Conclusions:
- Shadoo (Sho) deficiency does not influence the pathogenesis of prion disease in this mouse model.
- The reduction of Sho observed in prion disease may not play a critical role in disease progression.
- Further research is needed to fully elucidate the role of Sho in the nervous system and its potential involvement in other neurological conditions.
