Prions : The New Biology of Proteins

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ISBN: 9781420040128 | 142004012X
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Copyright: 12/20/2005

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Supplemental Materials

Each chapter ends with a concluding summation and references
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?
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Prions : The New Biology of Proteins

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Prions : The New Biology of Proteins

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