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Infectious prion diseases in humans: cannibalism, iatrogenicity and zoonoses

Stéphane Haïk1, Jean-Philippe Brandel1

  • 1Sorbonne Universités, UPMC Univ Paris 06 UMR S 1127, Inserm, U 1127, CNRS UMR 7225, ICM, F-75013 Paris, France; AP-HP, Groupe hospitalier Pitié-Salpêtrière, Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, F-75013 Paris, France; Centre National de Référence des Agents Transmissibles Non Conventionnels, F-75013 Paris, France.

Infection, Genetics and Evolution : Journal of Molecular Epidemiology and Evolutionary Genetics in Infectious Diseases
|June 24, 2014
PubMed

Insights

Human prion diseases, unlike other neurodegenerative disorders, are infectious. Strain properties, genetic factors, and transmission routes influence disease progression and characteristics, offering unique insights into prion propagation.

Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Protein Misfolding Disorders

Background:

  • Human prion diseases, such as kuru and Creutzfeldt-Jakob disease, are unique neurodegenerative disorders with infectious forms.
  • The transmissible agent is the abnormal prion protein isoform (PrPSc), which accumulates in the central nervous system.
  • PrPSc is characterized by beta-sheet enrichment, aggregation, and autocatalytic propagation.

Purpose of the Study:

  • To review and discuss the interplay of genetic factors, prion strain properties, and transmission routes in human prion diseases.
  • To analyze how these factors influence disease susceptibility, incubation period, and phenotypic expression.
  • To highlight the significance of infectious human prion diseases in understanding prion biology and strain selection.

Main Methods:

  • Review of existing literature on human prion diseases, including kuru, iatrogenic CJD, and variant CJD.
  • Analysis of transmission studies in experimental models to understand prion strain properties.
  • Examination of epidemiological data from historical epidemics (kuru, iatrogenic CJD, vCJD).

Main Results:

  • Conformational variations in PrPSc are believed to determine prion strain properties.
  • Genetic factors, transmission routes (e.g., cannibalism, medical procedures, diet), and strain characteristics collectively influence disease outcomes.
  • Historical epidemics provide crucial insights into prion disease dynamics in human populations.

Conclusions:

  • Infectious human prion diseases serve as a vital model for understanding prion strain selection and propagation.
  • The interaction between host genetics, prion strain, and exposure route dictates the clinical presentation and epidemiology of prion diseases.
  • Further research into these factors is essential for developing effective strategies against prion diseases.
Keywords:
Creutzfeldt-Jakob diseaseInfectious formsKuruPrion diseasesPrion strains

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