“Insights into Prion Biology:
Protein Misfolding in its Cellular Environment”
The prion hypothesis provides an explanation for a diverse collection of
previously inexplicable biological phenomena ranging from the
infectivity of neurodegenerative diseases, such as mad cow disease,
Creutzfeldt-Jakob disease, and scrapie, in mammals to the non-
Mendelian inheritance of unique traits in fungi. According to this idea,
prion-associated phenotypes arise when a protein adopts an alternative
conformation and persist when that form self-replicates. Determining
how the interplay between a prion misfolding pathway and its cellular
environment is balanced to create conformation-based phenotypes and
how it can be perturbed to “cure” these states is the major focus of my
research program.
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