Detection of Creutzfeldt-Jakob disease prions in skin: implications for healthcare

Akin Nihat,Simon Mead

doi:10.1186/s13073-018-0536-3

Abstract

Editorial summaryEvidence has recently been reported of prion seeding activity in skin tissue from patients with sporadic Creutzfeldt-Jakob disease (sCJD). This is relevant information for infection control measures during surgery. The work uses very sensitive prion assays now available for medical research, and may soon be adapted to related neurodegenerative disorders.

Highlights

Evidence has recently been reported of prion seeding activity in skin tissue from patients with sporadic Creutzfeldt-Jakob disease
Prion diseases Prion diseases are a group of incurable neurodegenerative disorders marked by the accumulation of misfolded forms of the normal cellular prion protein (PrP)
The most common human prion disease, sporadic Creutzfeldt-Jakob disease (sCJD), occurs at random in the population. This disease group is notorious for acquired forms: zoonotic variant Creutzfeldt-Jakob disease (CJD) arises following dietary exposure to bovine spongiform encephalopathy (BSE) prions; and iatrogenic CJD arises from exposure to prions as a result of a medical procedure

Summary

Introduction

Evidence has recently been reported of prion seeding activity in skin tissue from patients with sporadic Creutzfeldt-Jakob disease (sCJD). Evidence for prions in skin Animal bioassay is the only method to definitively demonstrate prion infectivity, but these are expensive and time-consuming experiments. Orrú and colleagues [1] demonstrated, for the first time, goldstandard evidence for the presence of human prions in skin, using a transmission study tuned to be sensitive: by intracerebral inoculation of inocula made from the skin of two patients with CJD, into mice that were engineered to express the human form of PrP.

Results

Conclusion