No Adaptation of the Prion Strain in a Heterozygous Case of Variant Creutzfeldt-Jakob Disease

Emerg Infect Dis. 2020 Jun;26(6):1300-1303. doi: 10.3201/eid2606.191116.

Abstract

We investigated a clinical case of variant Creutzfeldt-Jakob Disease in a person heterozygous for methionine/valine at codon 129 of the prion protein gene and identified the same strain properties in variant Creutzfeldt-Jakob disease in methionine homozygous persons and in bovine spongiform encephalopathy. These results indicate no adaptation of the agent in a different genetic background.

Keywords: Bovine spongiform encephalopathy; prions and related diseases; strain; transmissible spongiform encephalopathy; vCJD; variant Creutzfeldt-Jakob disease; zoonoses.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cattle
  • Codon
  • Creutzfeldt-Jakob Syndrome* / diagnosis
  • Creutzfeldt-Jakob Syndrome* / genetics
  • Encephalopathy, Bovine Spongiform*
  • Humans
  • Prion Proteins / genetics
  • Prions* / genetics

Substances

  • Codon
  • Prion Proteins
  • Prions