Localization of disease-related PrP in Danish patients with different subtypes of prion disease.
OBJECTIVE
The transmissible spongiform encephalopathies are characterized by vacuolization, neuronal loss, gliosis and deposition of a misfolded and Proteinase K resistant isoform of the prion protein (PrP(Sc)) in the central nervous system. METHODS MATERIALS AND PATIENTS: Paraffin-embedded tissue blot (PET-blot), immunohistochemistry (IHC) and Western blotting (WB) were combined to study the morphology and localization of disease related PrP in Danish patients with different subtypes of sporadic Creutzfeldt-Jakob disease, familiar Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease.


CONCLUSIONS
There was a good morphological and anatomical concordance between what was found with PET-blot and IHC in all patients. In some specific cases, the PET-blot was superior to IHC in sensitivity. To our knowledge, this is the first report where PET-blot analysis is applied to hereditary forms of human transmissible spongiform encephalopathies and compared with sporadic cases of Creutzfeldt-Jakob disease.
National Veterinary Institute, The Technical University of Denmark. anlb@lundbeck.com
A L
AL
Bergström
P M H
PM
Heegaard
H
H
Dyrbye
P
P
Lind
H
H
Laursen
D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D002531:Cerebellum / Q000378:metabolism / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D006224:Cricetinae; D003718:Denmark; D005625:Frontal Lobe / Q000378:metabolism / Q000473:pathology; D016098:Gerstmann-Straussler-Scheinker Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008647:Mesocricetus; D016612:Paraffin Embedding; D010784:Photomicrography; D018620:PrPSc Proteins / Q000235:genetics / Q000378:metabolism*; D011328:Prions / Q000378:metabolism*; D012160:Retina / Q000378:metabolism / Q000473:pathology; D017422:Sequence Analysis, DNA
D018620:PrPSc Proteins; D011328:Prions