Literature DB >> 10599800

Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain.

G Puoti1, G Giaccone, G Rossi, B Canciani, O Bugiani, F Tagliavini.   

Abstract

Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types of the protease-resistant form of the prion protein (type 1 and type 2 PrP(Sc)). We investigated 14 cases of sporadic CJD and found that both type 1 and type 2 PrP(Sc) coexisted in 5 subjects. The distinct PrP(Sc) isoforms were associated with different patterns of PrP deposition and severity of spongiform changes, suggesting that the PrP(Sc) type plays a central role in determining the neuropathologic profile of CJD.

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Year:  1999        PMID： 10599800     DOI： 10.1212/wnl.53.9.2173

Source DB:  PubMed          Journal:  Neurology        ISSN： 0028-3878            Impact factor:   9.910


  67 in total

1.  Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease.

Authors:  Piero Parchi; Maura Cescatti; Silvio Notari; Walter J Schulz-Schaeffer; Sabina Capellari; Armin Giese; Wen-Quan Zou; Hans Kretzschmar; Bernardino Ghetti; Paul Brown
Journal:  Brain       Date:  2010-09-07       Impact factor: 13.501

Review 2.  The prion strain phenomenon: molecular basis and unprecedented features.

Authors:  Rodrigo Morales; Karim Abid; Claudio Soto
Journal:  Biochim Biophys Acta       Date:  2006-12-15

3.  Diversity in prion protein oligomerization pathways results from domain expansion as revealed by hydrogen/deuterium exchange and disulfide linkage.

Authors:  Frederic Eghiaian; Thorsten Daubenfeld; Yann Quenet; Marieke van Audenhaege; Anne-Pascale Bouin; Guillaume van der Rest; Jeanne Grosclaude; Human Rezaei
Journal:  Proc Natl Acad Sci U S A       Date:  2007-04-18       Impact factor: 11.205

4.  Squirrel monkeys (Saimiri sciureus) infected with the agent of bovine spongiform encephalopathy develop tau pathology.

Authors:  P Piccardo; J Cervenak; O Yakovleva; L Gregori; K Pomeroy; A Cook; F S Muhammad; T Seuberlich; L Cervenakova; D M Asher
Journal:  J Comp Pathol       Date:  2011-10-20       Impact factor: 1.311

5.  Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics.

Authors:  Ignazio Cali; Rudolph Castellani; Amer Alshekhlee; Yvonne Cohen; Janis Blevins; Jue Yuan; Jan P M Langeveld; Piero Parchi; Jiri G Safar; Wen-Quan Zou; Pierluigi Gambetti
Journal:  Brain       Date:  2009-09-04       Impact factor: 13.501

6.  Incongruity between Prion Conversion and Incubation Period following Coinfection.

Authors:  Katie A Langenfeld; Ronald A Shikiya; Anthony E Kincaid; Jason C Bartz
Journal:  J Virol       Date:  2016-05-27       Impact factor: 5.103

Review 7.  Tracking protein aggregate interactions.

Authors:  Christina J Sigurdson; Jason C Bartz; K Peter R Nilsson
Journal:  Prion       Date:  2011-04-01       Impact factor: 3.931

8.  Experimental verification of a traceback phenomenon in prion infection.

Authors:  Atsushi Kobayashi; Nobuyuki Sakuma; Yuichi Matsuura; Shirou Mohri; Adriano Aguzzi; Tetsuyuki Kitamoto
Journal:  J Virol       Date:  2010-01-20       Impact factor: 5.103

9.  Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification.

Authors:  Piero Parchi; Rosaria Strammiello; Silvio Notari; Armin Giese; Jan P M Langeveld; Anna Ladogana; Inga Zerr; Federico Roncaroli; Patrich Cras; Bernardino Ghetti; Maurizio Pocchiari; Hans Kretzschmar; Sabina Capellari
Journal:  Acta Neuropathol       Date:  2009-08-29       Impact factor: 17.088

10.  Isolation of two distinct prion strains from a scrapie-affected sheep.

Authors:  Kentaro Masujin; Yujing Shu; Hiroyuki Okada; Yuichi Matsuura; Yoshifumi Iwamaru; Morikazu Imamura; Shirou Mohri; Takashi Yokoyama
Journal:  Arch Virol       Date:  2009-10-30       Impact factor: 2.574
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