Research Interests:
We are interested in the molecular pathogenesis of transmissible spongiform encephalopathies (TSEs), a family of fatal neurodegenerative disorders of humans and animals that includes Creutzfeldt-Jakob disease (CJD), bovine spongiform encephalopathy (BSE) or “mad cow disease”, and chronic wasting disease (CWD) of cervids. An essential component of the infectious agent is a misfolded, beta-sheet rich conformer of the cellular prion protein, thus the TSEs are also called prion diseases.
We are studying both the genetic and the environmental factors that influence prion transmission. Recently, we showed that mastitis can induce pathogenic prions to accumulate in the mammary gland. We are now exploring whether this environmental factor can enhance the transmission of prion disease via prion shedding into the milk. We are also investigating mutations in the prion gene that enable or inhibit prion transmission between species.
A third research focus is based on how the cellular prion protein structure may predispose to prion disease development. To this end, we have developed a transgenic mouse expressing a stucturally defined mutant prion protein. This mouse develops de novo prion disease, with prion aggregates and spongiform lesions in the brain, and may provide insights into the mechanisms of neurodegeneration and potential therapies to arrest a prion infection.
Track(s):
Molecular Pathology
Microbiology/Immunology
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