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Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System.

Katsuya Satoh1, Takayuki Fuse2, Toshiaki Nonaka2

  • 1Department of Locomotive Rehabilitation Science, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8501, Japan.

Molecules (Basel, Switzerland)
|January 1, 2020
PubMed
Summary

Human prion diseases, like Creutzfeldt-Jakob disease (CJD), show prion seeding activity in digestive organs. Esophageal activity in some CJD patients necessitates reconsidering endoscopic procedure safety.

Keywords:
prionrt-quic

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Area of Science:

  • Neurodegenerative diseases
  • Prion protein research
  • Infectious disease transmission

Background:

  • Human prion diseases are fatal neurodegenerative disorders linked to misfolded prion proteins.
  • Historically, prion infectivity was primarily associated with the central nervous system and lymphoreticular tissues.
  • Emerging evidence suggests prion seeding activity extends to non-neuronal organs like the liver, kidney, and skin.

Purpose of the Study:

  • To investigate the distribution of prion seeding activity in digestive organs of patients with sporadic and genetic human prion diseases.
  • To assess the potential risk of prion transmission during medical procedures involving affected tissues.

Main Methods:

  • Reanalysis of autopsy samples from patients diagnosed with sporadic and genetic human prion diseases.
  • Detection and quantification of prion protein seeding activity in various digestive organs.

Main Results:

  • Prion seeding activity was detected in nearly all examined digestive organs.
  • Esophageal prion activity in some Creutzfeldt-Jakob disease (CJD) patients was found to be comparable to levels in the central nervous system.
  • Significant prion seeding activity was observed across multiple gastrointestinal tissues.

Conclusions:

  • The widespread presence of prion seeding activity in the digestive tract, particularly the esophagus, challenges previous assumptions about prion disease localization.
  • Findings indicate a potential risk for prion transmission during endoscopic procedures, necessitating a reevaluation of safety protocols.
  • Further research is crucial to understand the implications of extraneural prion accumulation for patient safety and diagnostic procedures.