Prions and neurodegenerative diseases /

Detalles Bibliográficos
Clasificación:Libro Electrónico
Otros Autores: Legname, Giuseppe (Editor )
Formato: Electrónico eBook
Idioma:Inglés
Publicado: Cambridge, MA : Academic Press, 2020.
Colección:Progress in molecular biology and translational science ; v. 175.
Temas:
Prions.
Nervous system > Degeneration.
Prions (Virologie)
Nervous system > Degeneration
Prions
Acceso en línea:Texto completo
Tabla de Contenidos:
  • Intro
  • Prions and Neurodegenerative Diseases
  • Copyright
  • Contents
  • Contributors
  • Preface
  • Chapter One: Clinical diagnosis of human prion disease
  • 1. Introduction
  • 2. The diagnostic problem
  • 3. The non-specific supportive tests
  • 3.1. The EEG
  • 3.2. Brain MRI
  • 3.3. CSF protein tests
  • 3.4. Test combinations
  • 4. Specific tests: Detection of abnormal prion protein
  • 4.1. Capture/concentration techniques
  • 4.2. The development of protein ``amplification�� techniques
  • 5. Current routine clinical practice
  • 5.1. Brain biopsy
  • 5.2. PRNP analysis
  • 5.3. Tonsil biopsy
  • 5.4. CSF RT-QuIC
  • 6. Other tests
  • 6.1. Skin biopsy
  • 6.2. Nasal brushing/swabbing
  • 6.3. Urine tests
  • 6.4. Blood tests
  • 7. Concluding comments
  • References
  • Chapter Two: Understanding prion structure and conversion
  • 1. Introduction
  • 2. Structural analyses of infectious and non-infectious PrP aggregates
  • 3. Low-resolution approaches to study the structure of infectious prions
  • 4. Attempts to solve the structure of infectious prions at high-resolution
  • 5. Evolution of computational models for prion structure and conversion
  • 6. Conclusions
  • Acknowledgments
  • References
  • Chapter Three: Role of sialylation in prion disease pathogenesis and prion structure
  • 1. Introduction
  • 2. Diversity of N-glycans in PrPC and PrPSc
  • 3. N-glycan are exposed on a surface of PrPSc
  • 4. Sialylation status of PrPSc and prion infectivity
  • 5. Sialylation of PrPSc and lymphotropism
  • 6. Animal-to-human prion transmission
  • 7. Sialylation of PrPSc and neuroinflammation
  • 8. Structural constraints imposed by N-glycans
  • 9. Selective recruitment of PrPc sialoglycoforms and strain-specific structure
  • 10. Role of sialic acid residues and prion replication
  • 11. Sialylation and strain interference
  • 12. Concluding remarks
  • References
  • Chapter Four: Cofactor molecules: Essential partners for infectious prions
  • 1. Introduction
  • 2. Prion formation in vitro
  • 3. Isolation and identification of endogenous prion cofactors
  • 4. Cofactor molecules are required for the formation of infectious prions
  • 5. Cofactor molecules restrict the strain properties of purified prions
  • 6. Restoration of specific infectivity and strain properties from protein-only PrPSc
  • 7. Cofactor selection model of neurotropism.

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