Evidence map›Paper›PMID 25099577›Full record

ArticleThe New England journal of medicine2014

Prions in the urine of patients with variant Creutzfeldt-Jakob disease.

Fabio Moda, Pierluigi Gambetti, Silvio Notari, Luis Concha-Marambio, Marcella Catania, Kyung-Won Park, Emanuela Maderna, Silvia Suardi, Stéphane Haïk, Jean-Philippe Brandel and 4 more

Open access · bronzeAbstract read
In one paragraph

Article in The New England journal of medicine, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 95 papers.

0numbers the graph read from it
0cells of the map it votes in
95citing papers in PubMed
11.0field-weighted citation impact, top 1% 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

95 citing papers in PubMed, 211 citations in OpenAlex.

  1. Article
  2. Article
  3. Review
  4. Article
  5. Article
  6. Prion diseases: Lessons from historical outbreaks and potential emerging ones.Protein science : a publication of the Protein Society · 2025
    Review
  7. Article
  8. Review
  9. Review
  10. Diagnosis of Prion Diseases.Sub-cellular biochemistry · 2025
    Review
  11. Review
  12. Article
  13. Article
  14. Review
  15. Article
  16. Article
  17. Creutzfeldt-Jakob disease and other prion diseases.Nature reviews. Disease primers · 2024
    Review
  18. Article
  19. Review
  20. Review

35 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

14 authors at 7 institutions in 5 countries.

Fabio ModaFrom the Mitchell Center for Research in Alzheimer's Disease and Related Brain Disorders, University of Texas Medical School at Houston, Houston (F.M., L.C.-M., K.-W.P., C.S.); Foundation Carlo Besta Neurologic Institute, Milan (F.M., M.C., E.M., S.S., F.T.); National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland (P.G., S.N.); Universidad de los Andes, Facultad de Medicina, Santiago, Chile (L.C.-M.); Assistance Publique-Hôpitaux de Paris, Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Groupe Hospitalier Pitié-Salpêtrière, INSERM Unité 1127, Université Pierre et Marie Curie-Paris 6, and Centre Nationale de la Recherche Scientifique, Unité Mixte de Recherche - all in Paris (S.H., J.-P.B.); and the National CJD Research and Surveillance Unit, Western General Hospital, University of Edinburgh, Edinburgh (J.I., R.K.).
Pierluigi Gambetti
Silvio Notari
Luis Concha-Marambio
Marcella Catania
Kyung-Won Park
Emanuela Maderna
Silvia Suardi
Stéphane Haïk
Jean-Philippe Brandel
James Ironside
Richard Knight
Fabrizio Tagliavini
Claudio Soto
Fondazione IRCCS Istituto Neurologico Carlo Besta · ITCase Western Reserve University · USThe University of Texas Health Science Center at Houston · USWestern General Hospital · GBAssistance Publique – Hôpitaux de Paris · FRSorbonne Université · FRUniversidad de Los Andes, Chile · CL

Funding

Zoonotic potential of CWD and influence of environmental contamination on prion propagationP01AI077774 · NIAID · UNIVERSITY OF TEXAS MED BR GALVESTON · PI EDWARD Arthur HOOVER · 2008 to 2026
$36.1M
TRANSGENIC MODELS OF PRION DISEASES LINKED TO D178N MUTATIONP01AG014359 · NIA · CASE WESTERN RESERVE UNIVERSITY · PI GAMBETTI, PIERLUIGI · 1997 to 2013
$22.2M
Center for Clinical and Translational Sciences (CCTS)UL1TR000371 · NCATS · UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON · PI MCPHERSON, DAVID D · 2012 to 2016
$13.4M
Cyclic Amplification of Prion Protein MisfoldingR01NS049173 · NINDS · UNIVERSITY OF TEXAS MEDICAL BR GALVESTON · PI SOTO, CLAUDIO · 2005 to 2014
$3.2M
Absorption, Metabolism and Biodistribution of Prions after Oral IngestionR01NS078745 · NINDS · UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON · PI SOTO, CLAUDIO · 2012 to 2016
$2.2M
Development of a Biochemical Diagnosis for Creutzfeldt-Jakob diseaseR42NS079060 · NINDS · AMPRION, INC. · PI LEBOVITZ, RUSSELL M, SOTO, CLAUDIO · 2012 to 2014
$1.2M
Medical Research Council G0600953Medical Research Council G0900580NCATS NIH HHS UL1 TR000371NIAID NIH HHS P01 AI077774NIAID NIH HHS P01AI077774NIA NIH HHS P01 AG014359NIA NIH HHS P01AG14359NINDS NIH HHS R01 NS049173NINDS NIH HHS R01NS049173NINDS NIH HHS R01 NS078745NINDS NIH HHS R01NS078745NINDS NIH HHS R42 NS079060NINDS NIH HHS R42NS079060
6 · The paper itself

Abstract

backgroundPrions, the infectious agents responsible for transmissible spongiform encephalopathies, consist mainly of the misfolded prion protein (PrP(Sc)). The unique mechanism of transmission and the appearance of a variant form of Creutzfeldt-Jakob disease, which has been linked to consumption of prion-contaminated cattle meat, have raised concerns about public health. Evidence suggests that variant Creutzfeldt-Jakob disease prions circulate in body fluids from people in whom the disease is silently incubating.

methodsTo investigate whether PrP(Sc) can be detected in the urine of patients with variant Creutzfeldt-Jakob disease, we used the protein misfolding cyclic amplification (PMCA) technique to amplify minute quantities of PrP(Sc), enabling highly sensitive detection of the protein. We analyzed urine samples from several patients with various transmissible spongiform encephalopathies (variant and sporadic Creutzfeldt-Jakob disease and genetic forms of prion disease), patients with other degenerative or nondegenerative neurologic disorders, and healthy persons.

resultsPrP(Sc) was detectable only in the urine of patients with variant Creutzfeldt-Jakob disease and had the typical electrophoretic profile associated with this disease. PrP(Sc) was detected in 13 of 14 urine samples obtained from patients with variant Creutzfeldt-Jakob disease and in none of the 224 urine samples obtained from patients with other neurologic diseases and from healthy controls, resulting in an estimated sensitivity of 92.9% (95% confidence interval [CI], 66.1 to 99.8) and a specificity of 100.0% (95% CI, 98.4 to 100.0). The PrP(Sc) concentration in urine calculated by means of quantitative PMCA was estimated at 1×10(-16) g per milliliter, or 3×10(-21) mol per milliliter, which extrapolates to approximately 40 to 100 oligomeric particles of PrP(Sc) per milliliter of urine.

conclusionsUrine samples obtained from patients with variant Creutzfeldt-Jakob disease contained minute quantities of PrP(Sc). (Funded by the National Institutes of Health and others.).

Indexed as

Protein FoldingAdolescentAdultBrain ChemistryCreutzfeldt-Jakob SyndromeElectrophoresisHumansProtein EngineeringPrPSc ProteinsSensitivity and SpecificityYoung AdultPrPSc Proteins

Identifiers

PMID25099577
PMCPMC4162740
OpenAlexW2160881867

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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.