Description
Diseases such as CreutzfeldtJakob disease and kuru develop when certain 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 prion-like characteristics, and many of these are associated with important human diseases such as Alzheimer’s and 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 therapeutics. The contributors discuss how proteins such as tau, islet amyloid polypeptide, and a-synuclein aggregate, spread in a prion-like manner, and contribute to various neurodegenerative and extracerebral diseases. Human and animal diseases caused by the prion protein PrP are also covered, as are efforts to develop immunotherapeutics, protein disaggregases, and other drugs to delay or halt their progression. The authors discuss the development and use of cell culture and animal models of prion diseases, as well as the technologies and small molecules that are being used to study them. This volume is therefore useful for all biomedical scientists and physicians wishing to understand and treat this expanding group of devastating diseases.
