Localization of disease-related PrP in Danish patients with different subtypes of prion disease.

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 (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.

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.

• Authors: A L Bergström, P M H Heegaard, H Dyrbye, P Lind, H Laursen
• Journal: Clinical neuropathology (Clin Neuropathol) 2009 Sep-Oct Vol. 28 Issue 5 Pg. 321-32 ISSN: 0722-5091 [Print] Germany
• PMID: 19788047 (Publication Type: Journal Article)

Chemical References

• PrPSc Proteins
• Prions

Topics

• Animals
• Blotting, Western
• Brain (metabolism, pathology)
• Cerebellum (metabolism, pathology)
• Creutzfeldt-Jakob Syndrome (genetics, metabolism, pathology)
• Cricetinae
• Denmark
• Frontal Lobe (metabolism, pathology)
• Gerstmann-Straussler-Scheinker Disease (genetics, metabolism, pathology)
• Humans
• Immunohistochemistry
• Mesocricetus
• Paraffin Embedding
• Photomicrography
• PrPSc Proteins (genetics, metabolism)
• Prions (metabolism)
• Retina (metabolism, pathology)
• Sequence Analysis, DNA