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Updated: Aug 18, 2026

Procedures for Identifying Infectious Prions After Passage Through the Digestive System of an Avian Species
12:00

Procedures for Identifying Infectious Prions After Passage Through the Digestive System of an Avian Species

Published on: November 6, 2013

[Transmission of spongiform encephalopathies (prion diseases)]

P Federspil1, P A Federspil

  • 1Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätsklinken des Saarlandes, Kirrberger Strasse, 66421 Homburg/Saar. P.Federspil@uniklinik-saarland.de

HNO
|June 18, 2002
PubMed

Insights

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative disorders caused by misfolded prion proteins. This overview details their pathogenesis, epidemiology, and clinical features across various mammals.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Molecular Biology

Context:

  • Transmissible spongiform encephalopathies (TSEs), known as prion diseases, are fatal neurodegenerative conditions affecting the central nervous system.
  • These diseases are characterized by a distinctive spongiform histological pattern.

Purpose:

  • To provide a comprehensive overview of prion diseases.
  • To discuss the pathogenesis, epidemiology, and clinical manifestations of various TSEs.

Summary:

  • Prion diseases are caused by infectious agents, termed prions, which are pathologically folded forms of the normal prion protein (PrPC), designated PrPSc.
  • TSEs affect numerous mammals, including humans, with examples such as Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal familial insomnia (FFI), Kuru, and variant CJD (vCJD).
  • Notable animal TSEs include scrapie in sheep and bovine spongiform encephalopathy (BSE) in cattle.

Impact:

  • Enhances understanding of prion disease mechanisms and transmission.
  • Provides a foundational resource for researchers and clinicians studying neurodegenerative disorders.
  • Contributes to the knowledge base for managing and potentially treating prion-related illnesses.

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