NR AADK
AU Aguzzi,A.; Klein,M.A.; Musahl,C.; Raeber,A.J.; Blättler,T.; Hegyi,I.; Frigg,R.; Brandner,S.
TI Use of brain grafts to study the pathogenesis of prion diseases
QU Essays in Biochemistry 1998; 33: 133-47
PT journal article; review; review, tutorial
AB For the study of prion neurotoxicity, we used neural-grafting techniques: mice devoid of the normal host prion protein (Prnp% mice) received a neural graft and were intracerebrally infected with mouse prions. The growth and differentiation properties of neural grafts were defined. Growth of embryonic neuroectodermal tissue was optimal at gestational days 12.5-13.5. The blood-brain barrier is reconstituted after 7 weeks in most animals. Scrapie-infected PrPc-expressing grafts develop a severe spongiform encephalopathy and contain proteinase-resistant protein and infectivity. Infected grafts deliver high amounts of prions to the host brain without eliciting disease. Infected grafts show a progressive disruption of the blood-brain barrier. Following intraocular prion inoculation of a transplanted Prnp% mouse, prions do not reach the intracerebral graft, indicating that PrP expression is required for propagation along the optic tract.
ZR 35
MH Animal; Blood-Brain Barrier; Brain Tissue Transplantation; Central Nervous System/pathology; Disease Models, Animal; Fetal Tissue Transplantation; Human; Mice; Mice, Knockout; Prion Diseases/*etiology/pathology/transmission; Prions/genetics/pathogenicity; Scrapie/etiology/transmission; Support, Non-U.S. Gov't
AD Department of Pathology, University of Zürich, Switzerland.
SP englisch
PO England