Literature DB >> 12733425

Hereditary prion protein amyloidoses.

Bernardino Ghetti1, Fabrizio Tagliavini, M Takao, Orso Bugiani, Pedro Piccardo.   

Abstract

Prion protein (PrP) amyloid accumulation is the pathologic hallmark of some inherited prion diseases such as Gerstmann-Sträussler-Scheinker disease (GSS) and PrP cerebral amyloid angiopathy (PrP-CAA). In GSS, parenchymal amyloidosis may coexist with spongiform degeneration or neurofibrillary tangles, whereas in PrP-CAA, vascular amyloid coexists with neurofibrillary tangles. In GSS, N-truncated and C-truncated proteinase K-resistant PrP isoforms are present in the brain.

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Year:  2003        PMID： 12733425     DOI： 10.1016/s0272-2712(02)00064-1

Source DB:  PubMed          Journal:  Clin Lab Med        ISSN： 0272-2712            Impact factor:   1.935


  25 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.  Prions and the potential transmissibility of protein misfolding diseases.

Authors:  Allison Kraus; Bradley R Groveman; Byron Caughey
Journal:  Annu Rev Microbiol       Date:  2013-06-28       Impact factor: 15.500

3.  Proteolytic processing of the prion protein in health and disease.

Authors:  Hermann C Altmeppen; Berta Puig; Frank Dohler; Dana K Thurm; Clemens Falker; Susanne Krasemann; Markus Glatzel
Journal:  Am J Neurodegener Dis       Date:  2012-05-15

Review 4.  Micro-imaging of amyloid in mice.

Authors:  Jonathan S Wall; Michael J Paulus; Shaun Gleason; Jens Gregor; Alan Solomon; Stephen J Kennel
Journal:  Methods Enzymol       Date:  2006       Impact factor: 1.600

Review 5.  Current perspectives on cardiac amyloidosis.

Authors:  Jian Guan; Shikha Mishra; Rodney H Falk; Ronglih Liao
Journal:  Am J Physiol Heart Circ Physiol       Date:  2011-11-04       Impact factor: 4.733

6.  A novel PRNP-P105S mutation associated with atypical prion disease and a rare PrPSc conformation.

Authors:  E Tunnell; R Wollman; S Mallik; C J Cortes; S J Dearmond; J A Mastrianni
Journal:  Neurology       Date:  2008-10-28       Impact factor: 9.910

7.  Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP.

Authors:  Casper Jansen; Piero Parchi; Sabina Capellari; Ad J Vermeij; Patrizia Corrado; Frank Baas; Rosaria Strammiello; Willem A van Gool; John C van Swieten; Annemieke J M Rozemuller
Journal:  Acta Neuropathol       Date:  2009-11-13       Impact factor: 17.088

8.  [(11)C]PiB PET in Gerstmann-Sträussler-Scheinker disease.

Authors:  Kacie D Deters; Shannon L Risacher; Karmen K Yoder; Adrian L Oblak; Frederick W Unverzagt; Jill R Murrell; Francine Epperson; Eileen F Tallman; Kimberly A Quaid; Martin R Farlow; Andrew J Saykin; Bernardino Ghetti
Journal:  Am J Nucl Med Mol Imaging       Date:  2016-01-28

9.  Clinicopathological Correlates in a PRNP P102L Mutation Carrier with Rapidly Progressing Parkinsonism-dystonia.

Authors:  Chizoba C Umeh; Piyush Kalakoti; Michael K Greenberg; Silvio Notari; Yvonne Cohen; Pierluigi Gambetti; Adrian L Oblak; Bernardino Ghetti; Zoltan Mari
Journal:  Mov Disord Clin Pract       Date:  2016-02-18

Review 10.  Drosophila melanogaster as a model organism of brain diseases.

Authors:  Astrid Jeibmann; Werner Paulus
Journal:  Int J Mol Sci       Date:  2009-02-02       Impact factor: 6.208
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