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

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
• variant Creutzfeldt-Jakob disease
• transmissible spongiform encephalopathy
• zoonoses