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

A.L. Bergstrom; P.M. Heegaard; H. Dyrbye; P. Lind; H. Laursen

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

A.L. Bergstrom, P.M. Heegaard, H. Dyrbye, P. Lind, H. Laursen

BSc + MSc programme

Research output: Contribution to journal › Journal article › Research › peer-review

4 Citations (Scopus)

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-Straussler-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
Udgivelsesdato: 2009/9

Original language	English
Journal	Clinical Neuropathology
Volume	28
Issue number	5
Pages (from-to)	321-332
Number of pages	11
ISSN	0722-5091
Publication status	Published - 2009

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@article{8a973a0061ac11df928f000ea68e967b,

title = "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-Straussler-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 Udgivelsesdato: 2009/9",

author = "A.L. Bergstrom and P.M. Heegaard and H. Dyrbye and P. Lind and H. Laursen",

year = "2009",

language = "English",

volume = "28",

pages = "321--332",

journal = "Clinical Neuropathology",

issn = "0722-5091",

publisher = "Dustri-Verlag Dr. Karl Feistle",

number = "5",

}

TY - JOUR

T1 - Localization of disease-related PrP in Danish patients with different subtypes of prion disease

AU - Bergstrom, A.L.

AU - Heegaard, P.M.

AU - Dyrbye, H.

AU - Lind, P.

AU - Laursen, H.

PY - 2009

Y1 - 2009

N2 - 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-Straussler-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 Udgivelsesdato: 2009/9

AB - 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-Straussler-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 Udgivelsesdato: 2009/9

M3 - Journal article

SN - 0722-5091

VL - 28

SP - 321

EP - 332

JO - Clinical Neuropathology

JF - Clinical Neuropathology

IS - 5

ER -