Evidence map›Paper›PMID 42645584›Full record

ArticleActa neuropathologica2026

Distinctive properties of the prion protein in the brain and retina in the amyloidosis associated with the PRNP F198S mutation.

Bernardino Ghetti, Bradley S Glazier, Michele Fiorini, Kathy L Newell, José M Bonnin, Jill R Murrell, Leah Rie Varner, Max Jacobsen, James F Striebel, Suzette A Priola and 1 more

Abstract read
In one paragraph

Article in Acta neuropathologica, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

11 authors.

Bernardino GhettiDepartment of Pathology & Laboratory Medicine, Indiana University School of Medicine, 635 Barnhill Drive MS A138, Indianapolis, IN, 46202, USA. bghetti@iu.edu.
Bradley S GlazierDepartment of Pathology & Laboratory Medicine, Indiana University School of Medicine, 635 Barnhill Drive MS A138, Indianapolis, IN, 46202, USA.
Michele FioriniDepartment of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Piazzale L.A. Scuro, 10, 37134, Verona, Italy.
Kathy L NewellDepartment of Pathology & Laboratory Medicine, Indiana University School of Medicine, 635 Barnhill Drive MS A138, Indianapolis, IN, 46202, USA.
José M BonninDepartment of Pathology & Laboratory Medicine, Indiana University School of Medicine, 635 Barnhill Drive MS A138, Indianapolis, IN, 46202, USA.
Jill R MurrellDepartment of Pathology & Laboratory Medicine, Indiana University School of Medicine, 635 Barnhill Drive MS A138, Indianapolis, IN, 46202, USA.
Leah Rie VarnerRocky Mountain Laboratories, Laboratory of Neurological Infections and Immunity, National Institute of Allergy & Infectious Diseases, National Institutes of Health, 903 S. 4th Street, Hamilton, MT, 59840, USA.
Max JacobsenDepartment of Pathology & Laboratory Medicine, Indiana University School of Medicine, 635 Barnhill Drive MS A138, Indianapolis, IN, 46202, USA.
James F StriebelRocky Mountain Laboratories, Laboratory of Neurological Infections and Immunity, National Institute of Allergy & Infectious Diseases, National Institutes of Health, 903 S. 4th Street, Hamilton, MT, 59840, USA.
Suzette A PriolaRocky Mountain Laboratories, Laboratory of Neurological Infections and Immunity, National Institute of Allergy & Infectious Diseases, National Institutes of Health, 903 S. 4th Street, Hamilton, MT, 59840, USA.
Gianluigi ZanussoDepartment of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Piazzale L.A. Scuro, 10, 37134, Verona, Italy.

Funding

Research Education ComponentP30AG010133 · NIA · INDIANA UNIV-PURDUE UNIV AT INDIANAPOLIS · PI SAYKIN, ANDREW J · 1991 to 2020
$37.3M
Italian Ministry of Health RF 2013-02354884NIA NIH HHS P30 AG010133NIH/NIA P30 AG010133
6 · The paper itself

Abstract

Prion protein (PrP) deposits in the form of diffuse and cored plaques occur in most gray matter areas of the brain of individuals affected by Gerstmann-Sträussler-Scheinker disease (GSS) associated with the F198S mutation in PRNP; however, the PrP deposits in the retina have not been characterized. Furthermore, a comparative analysis of PrP deposits occurring in the brain and in the retina in GSS has not been carried out. We hypothesize that the PrP aggregation and seeding properties in the brain and retina differ. The aim of this study was to analyze PrP from brains and retinas of individuals affected by GSS, all carriers of the PRNP F198S mutation from the same pedigree. Postmortem tissues from these patients were analyzed using neuropathologic and biochemical methods including Real-Time Quaking Induced Conversion assay. PrP deposits in the retina were found only in the outer plexiform layer, did not have tinctorial property of amyloid and were immunopositive using nine antibodies recognizing epitopes throughout the PrP sequence. Biochemical analysis showed that PrP

Indexed as

AmyloidosisBrainGerstmann-Straussler-Scheinker DiseasePrion ProteinsPrionsRetinaAdultAgedFemaleHumansMaleMiddle AgedMutationPrion ProteinsPrionsPRNP protein, humanGerstmann–Sträussler–Scheinker diseasePrionPRNP F198S mutationPRNP geneRetina

Identifiers

PMID42645584
PMCPMC13518383

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