Fibrils in brain of Rocky Mountain elk with chronic wasting disease contain scrapie amyloid.
Chronic wasting disease (CWD), a progressive, fatal neurological disorder of captive mule deer and Rocky Mountain elk, is characterized neuropathologically by spongiform change in the neuropil, intraneuronal vacuolation and astrocytic hypertrophy and hyperplasia. Recently, scrapie amyloid-immunoreactive plaques have been demonstrated in brain tissues of CWD-affected captive mule deer, Rocky Mountain elk and hybrids of captive mule deer and white-tailed deer. We now report on the presence of abnormal fibrils isolated from brain tissues of Rocky Mountain elk using negative-stain electron microscopy. These fibrils resemble those found in scrapie-infected hamster brain. Furthermore, protein bands with relative molecular masses of 26 to 30 kilodaltons were shown to be immunoreactive to antibodies raised against scrapie amyloid by Western immunoblotting. Immuno-dot blot showed similar reactivity. Our data support the clinical and pathological diagnosis of the disease and provide further evidence that CWD belongs to the subacute spongiform encephalopathies.
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.
D C
DC
Guiroy
E S
ES
Williams
K J
KJ
Song
R
R
Yanagihara
D C
DC
Gajdusek
D000682:Amyloid / Q000378:metabolism*; D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology; D006224:Cricetinae; D003670:Deer; D004591:Electrophoresis, Polyacrylamide Gel; D015151:Immunoblotting; D007163:Immunosorbent Techniques; D008647:Mesocricetus; D008854:Microscopy, Electron; D009419:Nerve Tissue Proteins / Q000276:immunology; D009454:Neurofibrils / Q000378:metabolism* / Q000648:ultrastructure; D017096:Prion Diseases / Q000378:metabolism / Q000473:pathology / Q000662:veterinary*; D012608:Scrapie / Q000378:metabolism / Q000473:pathology*
D000682:Amyloid; D009419:Nerve Tissue Proteins