Creutzfeldt-Jakob Disease Prion Proteins in Human Brains

摘要

Creutzfeldt-Jakob disease is caused by a slow infectious pathogen, or prion. We found that purified fractions from the brain of two patients with CJD contained protease-resistant proteins ranging in apparent molecular weight from 10,000 to 50,000. These proteins reacted with antibodies raised against the scrapie prion protein PrP27-30. Rod-shaped particles were found in the brain tissue of the patients that were similar to those isolated from rodents with either scrapie or experimental Creutzfeldt-Jakob disease. After being stained with Congo red dye, the protein polymers from patients with CJD exhibited green birefringence when examined under polarized light. Our findings suggest that the amyloid plaques found in the brain of patients with CJD may be composed of paracrystalline arrays of prions similar to those in prion diseases in laboratory animals.