Diagnosis of Creutzfeldt-Jakob disease by Western blot identification of marker protein in human brain tissue.
We tested purified preparations of brain tissue from 39 patients with Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, or kuru, and from 32 patients with a variety of nonspongiform degenerative diseases, with the use of Western blots against an antiserum to a similarly purified fraction made from scrapie-infected hamster brain. Positive reactions occurred in 81 percent of the 31 specimens from the patients with Creutzfeldt-Jakob disease (and in all of the 7 specimens that were stored frozen for less than one year), in 3 of the 4 specimens from the patients with kuru, in 3 of the 4 specimens from the patients with Gerstmann-Sträussler-Scheinker syndrome, and in none of the specimens from the patients with other neurologic degenerative disorders, including familial or sporadic Alzheimer's disease; dementia associated with myoclonus, motor neuron disease, or parkinsonism; and acquired-immunodeficiency-syndrome encephalopathy. Immunologic testing has thus begun to provide a useful and rapid adjunct to neuropathological examinations and animal-transmission experiments for the diagnosis of the spongiform encephalopathies.
D000818:Animals; D001923:Brain Chemistry; D001927:Brain Diseases / Q000175:diagnosis; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis*; D006224:Cricetinae; D006801:Humans; D007122:Immunoelectrophoresis; D007729:Kuru / Q000175:diagnosis; D008854:Microscopy, Electron; D009419:Nerve Tissue Proteins / Q000276:immunology / Q000302:isolation & purification*; D009454:Neurofibrils / Q000032:analysis / Q000276:immunology; D011817:Rabbits; D012608:Scrapie / Q000276:immunology
D009419:Nerve Tissue Proteins