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Updated: Jul 13, 2026

Detection of Abnormal Prion Protein by Immunohistochemistry
Published on: May 5, 2023
[Neuropathological diagnosis of prion disease]
Shigeo Murayama1, Yuko Saito, Hiroyuki Hatsuta
1Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology and Tokyo Metropolitan Geriatric Hospital.
Abstract:
Neuropathological diagnosis of prion disease consists of sequence analysis of PRNP (prion protein gene), located on chromosome 20 and characterization and visualization of deposited proteinase K-resistant prion protein (PrP(Sc)). SNP at 129 locus (M/V) and Type 1 and Type 2 difference in Western blot analysis of PrP(Sc) from the postmortem brain influence the clinical and pathological presentations. Prion disease is classified into sporadic, hereditary and infectious subtypes, but PrP(Sc) from almost all the subtypes can transmit the disease to transgenic mice expressing human PRNP. Variant CJD, apparently derived from bovine spongiformic encephalopathy, requires shift in disease control strategy, in that PrP(Sc) is present in peripheral lymphatic organs.
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