NR ABKQ
AU Bockman,J.M.; Kingsbury,D.T.; McKinley,M.P.; Bendheim,P.E.; Prusiner,S.B.
TI Creutzfeldt-Jakob disease prion proteins in human brains
QU The New England Journal of Medicine 1985 Jan 10; 312(2): 73-8
PT journal article
AB Creutzfeldt-Jakob disease is caused by a slow infectious pathogen, or prion. We found that purified fractions from the brains of two patients with Creutzfeldt-Jakob disease 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 PrP 27-30. 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 Creutzfeldt-Jakob disease exhibited green birefringence when examined under polarized light. Our findings suggest that the amyloid plaques found in the brains of patients with Creutzfeldt-Jakob disease may be composed of paracrystalline arrays of prions similar to those in prion diseases in laboratory animals.
MH Adult; Aged; Animal; Birefringence; *Brain Chemistry; Case Report; Centrifugation, Density Gradient; Creutzfeldt-Jakob Syndrome/*metabolism; Female; Hamsters; Human; Male; Mice; Molecular Weight; *Prions; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.; Viral Proteins/immunology/*isolation & purification
SP englisch
PO USA
OR Prion-Krankheiten 2