Prions provides a well-organized overview of what is presently known about prion-related diseases.

Features:

• Presents an accurate description of human and animal prion-related diseases, plus broader implications of protein folding
• Describes the cell biology, genetic and putative function of prion proteins
• Examines the mechanisms of prion-related disease transmission
• Explores potential therapies, including the results of drug discovery
• Discusses applications of prion theories and concepts to other fields of health and biology

Contents

• Human and animal diseases: clinical symptoms, epidemiology, and neuropathology
• Human diseases
• Animal diseases
• Variant CJD
• Neuropathology
• The infectious agent and the prion hypothesis
• Hypothesis for the infectious agent
• Evidence supporting the prion hypothesis
• Criticisms of the prion hypothesis
• In vitro generation of prions
• The prion protein: structure, conversion, and mechanism of propagation
• Structural features of the cellular and scrapie prion protein isoforms
• Molecular mechanism of PrPC to PrPSc conversion
• Other factors involved in PrP conversion
• Peptide models used to understand PrP structure and conversion
• Cell biology, genetic and putative function of the normal prion protein
• Cellular biology of the normal prion protein
• A signaling role for the prion protein?
• PrP ligands indicate a potential role in apoptosis
• A putative role of PrP in copper metabolism
• PrP knockout animals and doppel
• Prion strains, species barriers, and multiple
• conformations of the prion protein
• Prion strains
• Species barrier
• Multiple conformations of PrP
• From the mouth to the brain
• Prions in the gastrointestinal tract
• The immune-system connection
• From the lymphoid organs to the brain: peripheral nerves or blood-brain barrier
• Neurodegeneration in prion diseases
• Characteristics of brain degeneration
• Is PrP the cause of TSE neurodegeneration
• Mechanism of neuronal apoptosis
• Neuronal apoptosis in TSEs involves the ER-stress pathway
• A role for the proteasome in TSE pathogenesis
• The diagnosis problem and current tests
• Importance of early diagnosis
• Difficulties of diagnosis
• Current status of TSE diagnosis in humans
• Postmortem detection of BSE in cattle
• The need for detection of PrP in blood
• Novel approaches under development for premortem early diagnosis
• Spectroscopic techniques
• Conformational antibodies
• PrP concentration by binding to specific ligands
• PrP amplification
• Therapeutic approaches
• Targets for TSE therapy
• Compounds under development for TSE treatment
• Immunization approach
• Cyclic amplification of prion protein misfolding: rationale, applications, and perspectives
• The rationale behind PMCA
• Applications of PMCA in prion diagnosis
• In vitro generation of infectious prions by PMCA
• Application of PMCA to understand the prion replication process
• Other diseases of protein misfolding
• Protein misfolding and disease
• Structural determinants of misfolding and aggregation
• Mechanism and driving forces in protein misfolding and aggregation
• Kinetics and intermediates of misfolding and aggregation
• Interactions between misfolded proteins
• Prions: a common phenomenon in biology
• The yeast prions
• The inherent infectious nature of misfolded aggregates
• Why are protein misfolding disorders other than TSE not infectious
• How common is the prion phenomenon in nature

Index

click here to see books � videos � cd-roms of related interest