[Neuropathological diagnosis of prion disease].
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.
Murayama
Shigeo
S
Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology and Tokyo Metropolitan Geriatric Hospital.
Saito
Yuko
Y
Hatsuta
Hiroyuki
H
Sakiyama
Yoshio
Y
D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002417:Cattle; D002890:Chromosomes, Human, Pair 20 / Q000235:genetics; D007562:Creutzfeldt-Jakob Syndrome; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D020641:Polymorphism, Single Nucleotide; D018620:PrPSc Proteins / Q000235:genetics / Q000378:metabolism; D017096:Prion Diseases / Q000145:classification / Q000175:diagnosis* / Q000473:pathology* / Q000635:transmission; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics
C512656:PRNP protein, human; D018620:PrPSc Proteins; D000072002:Prion Proteins; D011328:Prions