Investigating the Role of the Prion Protein Polybasic Domain in PrPSc Formation and Neuroprotection

Abstract

Prion diseases are characterized by the conformational conversion of the cellular prion protein (PrPC ) into the misfolded PrPSc isoform. However, both the normal, physiological function of PrPC and the mechanism of its conversion into PrPSc remain undefined. Several lines of evidence suggest that the expression of PrP can be neuroprotective, including its ability to abrogate the neurotoxic activity of PrP deletion mutants. In my thesis work, I characterized the role of the polybasic domain (residues 23-31) in the neuroprotective effect of WT PrP and examined the role of these residues in the formation of the infectious PrPSc protein.

Committee Chair

David Harris

Committee Members

Heather L. True-Krob, Kendall Blumer, David Holtzman, Jin-Moo Lee, Mark Sands

Comments

Permanent URL: https://doi.org/10.7936/K7639MPZ

Degree

Doctor of Philosophy (PhD)

Author's Department

Biology & Biomedical Sciences (Molecular Cell Biology)

Author's School

Graduate School of Arts and Sciences

Document Type

Dissertation

Date of Award

Summer 8-15-2011

Language

English (en)

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