Evidence map›Paper›PMID 26841849›Full record

ArticleScientific reports2016

Gerstmann-Sträussler-Scheinker disease subtypes efficiently transmit in bank voles as genuine prion diseases.

Laura Pirisinu, Michele A Di Bari, Claudia D'Agostino, Stefano Marcon, Geraldina Riccardi, Anna Poleggi, Mark L Cohen, Brian S Appleby, Pierluigi Gambetti, Bernardino Ghetti and 2 more

Open access · goldAbstract read
In one paragraph

Article in Scientific reports, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 47 papers.

0numbers the graph read from it
0cells of the map it votes in
47citing papers in PubMed
5.6field-weighted citation impact, top 4% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

47 citing papers in PubMed, 66 citations in OpenAlex.

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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

12 authors at 4 institutions in 2 countries.

Laura PirisinuDepartment of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
Michele A Di BariDepartment of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
Claudia D'AgostinoDepartment of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
Stefano MarconDepartment of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
Geraldina RiccardiDepartment of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
Anna PoleggiDepartment of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
Mark L CohenDepartment of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, School of Medicine, 2085 Adelbert Road Cleveland, Ohio, OH 44106, USA.
Brian S ApplebyDepartment of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, School of Medicine, 2085 Adelbert Road Cleveland, Ohio, OH 44106, USA.
Pierluigi GambettiDepartment of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, School of Medicine, 2085 Adelbert Road Cleveland, Ohio, OH 44106, USA.
Bernardino GhettiDepartment of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
Umberto AgrimiDepartment of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
Romolo NonnoDepartment of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
Istituto Superiore di Sanità · ITCase Western Reserve University · USIndiana University – Purdue University Indianapolis · USUniversity School · US

Funding

Research Education ComponentP30AG010133 · NIA · INDIANA UNIV-PURDUE UNIV AT INDIANAPOLIS · PI SAYKIN, ANDREW J · 1991 to 2020
$37.3M
Neuropathology Core - P GambettiP01AI106705 · NIAID · CASE WESTERN RESERVE UNIVERSITY · PI GAMBETTI, PIERLUIGI · 2014 to 2018
$7.7M
Structural correlates of prion strain, phenotype and infectivity in human prion dR01NS083687 · NINDS · CASE WESTERN RESERVE UNIVERSITY · PI GAMBETTI, PIERLUIGI, SUREWICZ, WITOLD K · 2014 to 2018
$2.9M
Pathogenicity of prion protein aggregates from uninfected cells and brainsR01NS062787 · NINDS · CASE WESTERN RESERVE UNIVERSITY · PI ZOU, WEN-QUAN · 2009 to 2013
$1.4M
NIAID NIH HHS AI106705NIAID NIH HHS P01 AI106705NIA NIH HHS P30 AG010133NIA NIH HHS P30AG010133NINDS NIH HHS NS062787NINDS NIH HHS R01 NS062787NINDS NIH HHS R01 NS083687
6 · The paper itself

Abstract

Gerstmann-Sträussler-Scheinker disease (GSS) is an inherited neurodegenerative disorder associated with mutations in the prion protein gene and accumulation of misfolded PrP with protease-resistant fragments (PrP(res)) of 6-8 kDa. With the exception of a few GSS cases characterized by co-accumulation of PrP(res) of 21 kDa, efforts to transmit GSS to rodents have been unsuccessful. As a result, GSS subtypes exclusively associated with 6-8 kDa PrP(res) have often been considered as non-transmissible proteinopathies rather than true prion diseases. We show that GSS with P102L, A117V and F198S mutations transmit efficiently and produce distinct pathological phenotypes in bank voles (M. glareolus), irrespective of the presence of 21 kDa PrP(res) in the inoculum, demonstrating that GSS is a genuine prion disease characterized by both transmissibility and strain variation.

Indexed as

MutationAnimalsArvicolinaeDisease Models, AnimalGerstmann-Straussler-Scheinker DiseaseHumansPrion ProteinsPrion Proteins

Identifiers

PMID26841849
PMCPMC4740801
OpenAlexW2409100080

What OpenQuestion holds

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LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.