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Biotechnology Book from C.H.I.P.S.

Prions
New Biology of Proteins
by Claudio Soto

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

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Prions
New Biology of Proteins
by Claudio Soto

2005 184 pages $148.95 + shipping
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