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.
Guiroy
D C
DC
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.
Williams
E S
ES
Song
K J
KJ
Yanagihara
R
R
Gajdusek
D C
DC
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