NR ABUU
AU Brown,P.; Kenney,K.; Little,B.; Ironside,J.; Will,R.; Cervenakova,L.; Bjork,R.J.; San Martin,R.A.; Safar,J.G.; Roos,R.; Haltia,M.; Gibbs,C.J.Jr.; Gajdusek,D.C.
TI Intracerebral distribution of infectious amyloid protein in spongiform encephalopathy
QU Annals of Neurology 1995 Aug; 38(2): 245-53
PT journal article
AB We studied the regional distribution of infectious amyloid protein by western immunoblots of brain tissue extracts from 37 patients with different forms of spongiform encephalopathy, i.e., 16 sporadic cases, 18 familial cases with a variety of mutations, and 3 iatrogenic cases. In sporadic and familial Creutzfeldt-Jakob disease, amyloid protein concentrations were usually highest in the frontotemporal regions of the cerebral cortex, whereas iatrogenic Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker syndrome had as high or higher concentrations in the deep cerebral nuclei and cerebellum. As a group, familial cases had lower amyloid protein concentrations than either sporadic or iatrogenic cases, and fatal familial insomnia patients had the lowest concentrations found in any form of disease. This hierarchy of amyloid protein concentrations corresponds to the experimental transmission rates observed for each form of disease and is consistent with the concept that the protein molecule is an integral component of the infectious agent. Regional amyloid protein pattern analysis of brain and spinal cord may help to distinguish sporadic from environmentally acquired infections, as for example, cases of human disease suspected to have arisen from exposure to sheep or cows infected with scrapie or bovine spongiform encephalopathy.
IN Mittels Western Blots von Proben aus verschiedenen Regionen 37 menschlicher Gehirne wurden Unterschiede zwischen spongiformen Enzephalopathien verschiedenen Ursprunges gefunden. Bei sogenannten sporadischen und erblichen Creutzfeldt-Jakob-PatientInnen war die Konzentration der Amyloide im Bereich der Stirn und Schläfen am größten. Dagegen waren die Amyloidkonzentrationen bei eindeutig durch medizinische Maßnahmen infizierten Creutzfeldt-Jakob-PatientInnen und Gerstmann-Sträussler-Scheinker-PatientInnen im Kleinhirn und tief im Großhirn mindestens ebenso groß. Die erblichen Formen, insbesondere die tödliche familiäre Schlaflosigkeit, führten generell zu geringeren Amyloidkonzentrationen als die iatrogenen und die scheinbar spontanen. Diese Unterschiede bei den Amyloidkonzentrationen korrespondieren mit unterschiedlichen Infektiositäten.
ZR 23 Zitate
MH Aged; Amyloid/genetics/*metabolism; Case Report; Cerebral Cortex/*metabolism/pathology; Codon; Creutzfeldt-Jakob Syndrome/genetics/*metabolism/pathology; Female; Gerstmann-Sträussler-Scheinker Disease/genetics/*metabolism/pathology; Human; Male; Middle Age; Polymorphism (Genetics); Prion Diseases/genetics/*metabolism/pathology; Support, Non-U.S. Gov't
AD Dr Paul Brown, MD, L. Cervenáková, PhD, K. Kenney, MD, R. A. San Marchtin, MD, J. Safar, MD, C. J. Gibbs Jr., PhD, D. C. Gajdusek, MD, Laboratory of CNS Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA; B. Little, MD, Department of Pathology, Lehigh Valley Medical Center, Allentown, PA; J. Ironside, MRCPath, R. Will, MD, CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland; R. J. Bjork, MD, Colorado Springs Neurological Associates, Colorado Springs, CO; R. Roos, MD, Department of Neurology, University of Chicago Medical Center, Chicago, IL; M. Haltia, MD, Department of Pathology, University of Helsinki, Helsinki, Finland; Correspondence to P. Brown, Building 36, Room 5B21, National Institutes of Health, Bethesda, MD 20892
SP englisch
PO USA