Localization of disease-related PrP in Danish patients with different subtypes of prion disease
1 National Veterinary Institute, The Technical University of Denmark, and
2 The Neuropathology Laboratory, Rigshospitalet, Copenhagen, Denmark
DOI 10.2379/NPX07098
Abstract
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 (PrPSc) 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. Results and conclusion: 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.
Author Details
Authors
Departments
- 1 National Veterinary Institute, The Technical University of Denmark, and
- 2 The Neuropathology Laboratory, Rigshospitalet, Copenhagen, Denmark
Address
A.-L. Bergström, PhD, National Veterinary Institute, The Technical University of Denmark, Bülowsvej 27, 1790 Copenhagen V, Denmark
Citation
R.J. Desnick, M. Banikazemi and M. Wasserstein. Localization of disease-related PrP in Danish patients with different subtypes of prion disease. ; : 0--1. doi: 10.2379/NPX07098.
