Medicine (RMH) - Theses

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    Expanding our understanding of the clinico-epidemiology, cellular biology and pathogenesis of human prion diseases
    Collins, Steven John ( 2021)
    This thesis primarily constitutes a compendium of published manuscripts encapsulating a substantial volume of work relating to prion diseases, undertaken from 1999 to 2020 through the Australian National Creutzfeldt-Jakob Disease Registry based at the University of Melbourne, frequently in collaboration with pre-eminent domestic and international scholars in the field of prion diseases. The thesis is divided into two principal parts. The first comprises 78 peer-reviewed papers submitted in full, representing a diverse array of acclaimed, often landmark or pivotal, studies published in prestigious general medical and specialist journals where I have been either senior and corresponding author, primary author or leader of the Australian contribution in major multi-national projects. This body of work is divided into seven sections spanning: normal prion protein cellular biology; prion pathogenesis; human prion disease epidemiological studies; human prion disease bio-marker studies; clinical aspects of human prion disease; prion disease treatment studies; and studies of human prion-like diseases. A short review at the start of the thesis and a preamble before each of these sections dealing with my principal publications are offered to place these published studies in the context of extant clinical and scientific literature. The second part of the thesis is submitted for completeness, comprising four sub-sections encompassing 61 publications and reports, listed by title and publication details, dealing with human prion disease for which I was a significant contributor.