{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T06:58:38Z","timestamp":1773212318715,"version":"3.50.1"},"reference-count":37,"publisher":"The Royal Society","issue":"1306","license":[{"start":{"date-parts":[[1994,3,29]],"date-time":"1994-03-29T00:00:00Z","timestamp":764899200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/royalsociety.org\/journals\/ethics-policies\/data-sharing-mining\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1994,3,29]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Recent molecular genetic studies revealed that the human prion protein (PrP) gene has a large repertoire of polymorphisms and mutations. Each variant PrP seems to correspond to a distinct type of prion diseases. W e report herein that it is useful to classify prion diseases into plaque type or non-plaque type, based on the distribution of PrP in the central nervous system. The variant PrP including codon 102, codon 105, codon 129, codon 145 and insertional polymorphisms belong to the plaque type prion diseases, whereas the wild-type PrP and the variants including codon 180, codon 200, and codon 232 polym orphisms belong to the non-plaque type. T he non-plaque type prion diseases showed a rapidly progressive dem entia, myoclonus and periodic synchronous discharges in the electroencephalogram , and in the pathological findings diffuse grey matter PrP accumulations including the synaptic structures. The plaque type prion diseases showed a long clinical course without myoclonus and periodic synchronous discharges, and the major PrP accumulation sites were extracellular PrP plaques. The distribution of PrP deposits in the central nervous system influences the clinical and pathological aspects of prion diseases. Thus, PrP accumulations may play a central role in the pathogenesis of prion diseases.<\/jats:p>","DOI":"10.1098\/rstb.1994.0034","type":"journal-article","created":{"date-parts":[[2006,12,15]],"date-time":"2006-12-15T17:54:58Z","timestamp":1166205298000},"page":"391-398","source":"Crossref","is-referenced-by-count":74,"title":["Human prion diseases with variant prion protein"],"prefix":"10.1098","volume":"343","author":[{"given":"Tetsuyuki","family":"Kitamoto","sequence":"first","affiliation":[{"name":"Department of Neuropathology, Neurological Institute, Kyushu University, Fukuoka 812, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun","family":"Tateishi","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Neurological Institute, Kyushu University, Fukuoka 812, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"175","reference":[{"key":"p_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00427210"},{"key":"p_2","first-page":"974","article-title":"P ro -L eu change at position 102 of prion protein is the most com m on b u t not the sole m u tatio n related to G erstm ann-S traussler syndrom e. 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U.S.A."},{"key":"p_9","first-page":"806","article-title":"F ata l fam ilial insom nia an d fam ilial C reu tzfeld t-Ja k o b disease: disease p h en o ty p e d eterm in ed by a D N A polym orphism . Science","volume":"258","author":"Petersen R.B.","year":"1992","journal-title":"Wash."},{"key":"p_10","unstructured":"G o Idgaber D . G oldfarb L .G . Brow n P. et at. 1989 M u tatio n s in fam ilial C reu tzfeld t-Ja k o b disease and G erstm a n n -S trau ssler syndrom e. Expl. Neurol. 106 2 04-206."},{"key":"p_11","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(93)90275-4"},{"key":"p_12","first-page":"342","article-title":"L inkage o f a prion p ro tein missense v aria n t to G erstm a n n -S trau ssler syndrom e. Nature","volume":"338","author":"Baker H .","year":"1989","journal-title":"Lond."},{"key":"p_13","doi-asserted-by":"crossref","unstructured":"H siao K . D louhy S .R . Farlow M .R . et at. 1992 M u ta n t prion proteins in G erstm a n n -S trau ssler-S c h ein k e r disease w ith neurofibrillary tangles. Nature genet. 1 68-71.","DOI":"10.1038\/ng0492-68"},{"key":"p_14","first-page":"1587","article-title":"S pontaneous neuro d eg en eratio n in transgenic mice w ith m u ta n t prion p rotein of G erstm an n -S trau ssler syndrom e. Science","volume":"250","author":"Scott M .","year":"1990","journal-title":"Wash."},{"key":"p_15","doi-asserted-by":"crossref","unstructured":"Inoue I. K itam o to T . D oh-ura K . Shii H . G oto I. T ateishi J . 1994 Ja p an e se family o f C reu tzfeld t-Jak o b disease w ith codon 200 point m u tatio n o f the prion protein gene. Neurology. 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