Preparation of lyophilized recombinant prion protein for TSE diagnosis by RT-QuIC

摘要

ObjectiveTransmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative diseases, often referred as prion diseases. TSEs result from the misfolding of the cellular prion protein (PrP^C) into a pathogenic form (PrP^Sc) that accumulates in the brain and lymphatic tissue. Amplification based assays such as real-time quaking induced conversion allow us to assess the conversion of PrP^C to PrP^Sc. Real-time quaking induced conversion (RT-QuIC) can be used for the detection of PrP^Sc in a variety of biological tissues from humans and animals. However, RT-QuIC requires a continuous supply of freshly purified prion protein and this necessity is not sustainable in a diagnostic laboratory setting.