NR AHGP
AU Liberski,P.P.; Guiroy,D.C.; Williams,E.S.; Walis,A.; Budka,H.
TI Deposition patterns of disease-associated prion protein in captive mule deer brains with chronic wasting disease
QU Acta Neuropathologica 2001 Nov; 102(5): 496-500
PT journal article
AB Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) in captive and free-ranging cervids in the USA; its origin is obscure. Archival formalin-fixed and paraffin-embedded specimens of 16 captive mule deer brains with CWD were analyzed using immunocytochemistry for the disease-associated prion protein (PrP). The most prominent pattern of PrP deposition were plaque-like structures, a substantial proportion of which were florid plaques surrounded by a rim of spongiform vacuoles. The percentage of florid plaques was highly variable according to region, ranging from 0% to 52.7%. The highest percentage was observed in the medulla and basal ganglia, the lowest in the cerebral cortex. Only three brains contained no florid plaques. There were also punctate synaptic-type and perivascular deposits, particularly in areas of severe spongiform change, and subpial and subependymal plaque-like deposits, whereas cerebellar involvement was mild. Thus, CWD brain pathology prominently features florid PrP plaques, as does variant Creutzfeldt-Jakob disease (vCJD), but differs in other characteristics from vCJD.
MH Amino Acid Sequence; Animal; Brain/*pathology; Deer; Human; Molecular Sequence Data; Prion Diseases/*veterinary; Prions/*analysis; Support, Non-U.S. Gov't
AD Institute of Neurology, University of Vienna, Wien, Austria.
SP englisch
PO Deutschland