NR AAZW
AU Baron,H.; Baron-van Evercooren,A.; Brucher,J.M.
TI Antiserum to scrapie-associated fibril protein reacts with amyloid plaques in familial transmissible dementia
QU Journal of Neuropathology and Experimental Neurology 1988 Mar; 47(2): 158-65
ER J Neuropathol Exp Neurol 1988 Jul;47(4):496
PT journal article
AB Scrapie-associated fibrils (SAF) are disease-specific markers for the unconventional agent-induced, transmissible spongiform encephalopathies. Polyclonal rabbit antiserum to SAF protein was reacted with brain sections from scrapie-infected mice, two familial cases of transmissible dementia, and three cases of Alzheimer's disease (AD). Specific immunostaining of cerebral amyloid plaques occurred in the scrapie-infected mice and in the two familial cases of transmissible dementia. No immunoreactivity was detected in senile plaques or neurofibrillary tangles in the three cases of AD. Our results suggest that SAF, the causative pathogenic agent, and extracellular deposits of amyloid in the brain are closely related. Immunohistochemical detection of SAF protein could serve as a useful diagnostic adjunct in the postmortem evaluation of difficult cases of dementia. The identification of SAF protein in the brains of two affected members of a family combining the clinical and pathological features of Creutzfeldt-Jakob disease (CJD) and the Gerstmann-Sträussler syndrome (GSS) substantiates earlier conclusions of a nosological relationship between the two. Our study provides further evidence of the similarity of SAF protein to prion protein (PrP 27-30).
MH Adult; Amyloid/*immunology; Creutzfeldt-Jakob Syndrome/metabolism/pathology; Dementia/*genetics/immunology; France; Human; Immune Sera/immunology; Immunohistochemistry; Male; Middle Age; Nerve Tissue Proteins/*immunology; PrP 27-30 Protein; Slow Virus Diseases/metabolism/pathology; Support, Non-U.S. Gov't
AD Laboratoire de Neurovirologie, Hopital de la Salpetriere, Paris, France.
SP englisch
PO USA