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Presents an accurate description of prion diseases, describing the science, concepts, hypothesis, and mechanisms of prion disease transmission. It covers human and animal prion diseases, their incidence, prevalence, origin, and clinical and neuropathologic characteristics.
Each chapter ends with a concluding summation and referencesHuman and animal diseases: clinical symptoms, epidemiology, and neuropathologyHuman diseasesAnimal diseasesVariant CJDNeuropathologyThe infectious agent and the prion hypothesisHypothesis for the infectious agentEvidence supporting the prion hypothesisCriticisms of the prion hypothesisIn vitro generation of prionsThe prion protein: structure, conversion, andmechanism of propagationStructural features of the cellular and scrapie prion protein isoformsMolecular mechanism of PrPC to PrPSc conversionOther factors involved in PrP conversionPeptide models used to understand PrP structure and conversionCell biology, genetic and putative function of the normal prion proteinCellular biology of the normal prion proteinA signaling role for the prion protein?PrP ligands indicate a potential role in apoptosisA putative role of PrP in copper metabolismPrP knockout animals and doppelPrion strains, species barriers, and multipleconformations of the prion proteinPrion strainsSpecies barrierMultiple conformations of PrPFrom the mouth to the brainPrions in the gastrointestinal tractThe immune-system connectionFrom the lymphoid organs to the brain: peripheral nerves or blood-brain barrier?Neurodegeneration in prion diseasesCharacteristics of brain degenerationIs PrP the cause of TSE neurodegeneration?Mechanism of neuronal apoptosisNeuronal apoptosis in TSEs involves the ER-stress pathwayA role for the proteasome in TSE pathogenesis?The diagnosis problem and current testsImportance of early diagnosisDifficulties of diagnosisCurrent status of TSE diagnosis in humansPostmortem detection of BSE in cattleThe need for detection of PrP in bloodNovel approaches under development for premortem early diagnosisSpectroscopic techniquesConformational antibodiesPrP concentration by binding to specific ligandsPrP amplificationTherapeutic approachesTargets for TSE therapyCompounds under development for TSE treatmentImmunization approachCyclic amplification of prion protein misfolding: rationale, applications, and perspectivesThe rationale behind PMCAApplications of PMCA in prion diagnosisIn vitro generation of infectious prions by PMCAApplication of PMCA to understand the prion replication processOther diseases of protein misfoldingProtein misfolding and diseaseStructural determinants of misfolding and aggregationMechanism and driving forces in protein misfolding and aggregationKinetics and intermediates of misfolding and aggregationInteractions between misfolded proteinsPrions: a common phenomenon in biology?The yeast prionsThe inherent infectious nature of misfolded aggregatesWhy are protein misfolding disorders other than TSE not infectious?How common is the prion phenomenon in nature?
