New Scholar Award in Aging
Jiyan Ma, Ph.D.
Ohio State University

The Pathogenic Mechanism of Prion Disease

Prion diseases belong to a large group of neurodegenerative disorders occurring in the elderly, such as Alzheimerís disease and Parkinsonís disease. Like other neurodegenerative disorders, prion disease can be manifested as a sporadic or inherited disease. However, prion diseases can also be infectious. This characteristic distinguishes the prion disease from other age-related neurodegenerative diseases.

The prion protein, called PrP, has been proven to be involved in the pathogenesis of prion disease. During the disease, the normal PrP, referred to as PrPC, accumulates in a different conformation, referred to as PrPSc. The PrPSc form is aggregated and partially resistant to protease digestion. The prion hypothesis postulates that PrPSc is the infectious agent. During the disease, PrPSc coerces the normal PrPC to adopt this pathogenic conformation.

Despite all the effort, it is still not clear why the neurons die during prion disease. We recently have identified that when PrP is in cytosol, the abnormal compartment for its location in the cell, it is extremely neurotoxic. We are investigating the relationship among cytosolic PrP, aggregation and conformational change of PrP, neurotoxicity and the influence of aging on these processes. The long-term goal of our research is to understand the pathogenic mechanism, and to develop preventive and therapeutic methods of these fatal neurodegenerative diseases.


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