Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System

Molecules. 2019 Dec 16;24(24):4601. doi: 10.3390/molecules24244601.

Abstract

Human prion diseases are neurodegenerative disorders caused by prion protein. Although infectivity was historically detected only in the central nervous system and lymphoreticular tissues of patients with sporadic Creutzfeldt-Jakob disease, recent reports suggest that the seeding activity of Creutzfeldt-Jakob disease prions accumulates in various non-neuronal organs including the liver, kidney, and skin. Therefore, we reanalyzed autopsy samples collected from patients with sporadic and genetic human prion diseases and found that seeding activity exists in almost all digestive organs. Unexpectedly, activity in the esophagus reached a level of prion seeding activity close to that in the central nervous system in some CJD patients, indicating that the safety of endoscopic examinations should be reconsidered.

Keywords: prion; rt-quic.

MeSH terms

  • Adult
  • Aged
  • Case-Control Studies
  • Creutzfeldt-Jakob Syndrome / metabolism
  • Creutzfeldt-Jakob Syndrome / pathology
  • Digestive System / metabolism*
  • Digestive System / pathology*
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Organ Specificity
  • Postmortem Changes
  • Prion Diseases / metabolism*
  • Prion Diseases / pathology*
  • Prions / metabolism*

Substances

  • Prions

Supplementary concepts

  • Creutzfeldt-Jakob Disease, Sporadic