Prion diseases, also known as Transmissible Spongiform Encephalopathies
(TSEs), exist in both humans (Creutzfeldt-Jakob disease (CJD)) and
animals (scrapie, bovine spongiform encephalopathy (BSE), chronic
wasting disease) and have the unique property of being infectious,
sporadic or genetic in origin.
Although the precise nature of the infectious agent responsible for TSEs
is not definitely identified, it is now clearly demonstrated that a
protein named PrP (for Prion Protein) plays a critical role in the
transmission and pathogenesis of TSEs.
This book provides the general description as well as the details of the
techniques currently used for the study of prion diseases. Taking into
account the pivotal role played by PrP it is not surprising that many
Chapters of this book deal with the purification, the detection and the
characterization of the different forms of this protein. In addition, in
vitro, cellular and animal models specifically adapted to the study of
TSEs, as well as bio-safety procedures are described. Each Chapter is
written by scientists involved for many years in their respective domain
of prion biology who give the best of their knowledge in this technical
document. This volume is a very useful tool for any laboratory which
recently decided to contribute to the study of TSEs as well as for teams
already engaged in this field for many years but interested in extending
their technical capacity toward new methods.
Features:
Purification of PrPC and the Pathological Isoform of Prion Protein
(PrPsc or PrP-res)
Animal Models of TSEs
Cell Culture Models of TSEs
PrPsc Immunohistochemistry
Western Immunoblotting Techniques
Antibody Production and ELISA
TSE Strain Typing in Mice
Biosafety and Decontamination Procedures
Cell-free Conversion of Prion Proteins
Cytotoxicity of PrP Peptides
Cyclic Amplification of Prion Protein Misfolding
Of interest to:
Researchers and clinicians in the fields of cell biology, biomedicine,
neuroscience/neuropathology, veterinary medicine and biochemistry.
