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ABSTRACT
Prions are infectious proteins and over the past few decades, some prions have become renowned for their causative role in several neurodegenerative diseases in animals and humans. Since their discovery, the mechanisms and mode of transmission and molecular structure of prions have begun to be established. There is, however, still much to be elucidated about prion diseases, including the development of potential therapeutic strategies for treatment. The significance of prion disease is discussed here, including the categories of human and animal prion diseases, disease transmission, disease progression and the development of symptoms and potential future strategies for treatment. Furthermore, the structure and function of the normal cellular prion protein (PrPC) and its importance in not only in prion disease development, but also in diseases such as cancer and Alzheimer's disease will also be discussed.
Funding
This work was supported by grants from the Australian Research Council (DP110100389) to ALM. Molecular Basis of Disease Program of the Menzies Health Institute Strategic Project Grant to Trina Stewart and ALM.