Prion Diseases

Diseases such as Creutzfeldt-Jakob disease and kuru develop when PrP proteins form prions by misfolding, clumping together, and spreading from cell to cell. Over the past decade, a number of proteins have been reported to possess the characteristics of PrP prions. A growing collection of disorders has been found to be caused by prions; some of these prion diseases include such devastating illnesses as Alzheimer’s and Parkinson’s and possibly type 2 diabetes. Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Medicine covers the progress that has recently been made in our understanding of the pathophysiology of prion diseases, as well as ongoing efforts to develop effective therapeutics. The contributors discuss how proteins such as tau, islet amyloid polypeptide, and alpha-synuclein adopt alternative shapes that lead them to aggregate, resulting in cellular degeneration. Therapies for human and animal diseases caused by prions are also covered. This volume is therefore useful for all biomedical scientists and physicians wishing to understand and treat this expanding group of devastating disorders.