Evidence map›Paper›PMID 29473169›Full record

ReviewJournal of neurochemistry2018

Animal models of Wilson disease.

Emily Reed, Svetlana Lutsenko, Oliver Bandmann

Open access · bronzeAbstract readReview
In one paragraph

Review in Journal of neurochemistry, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
30citing papers in PubMed, 1 pooled it
3.3field-weighted citation impact, top 8% 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

30 citing papers in PubMed, 1 synthesis or guideline pooled it, 54 citations in OpenAlex.

  1. Pooled it
  2. Review
  3. Some Properties of theInternational journal of molecular sciences · 2025
    Article
  4. Article
  5. Effect of primary copper metabolism disturbance on elemental, protein, and lipid composition of the organs in Jackson toxic milk mouse.Biometals : an international journal on the role of metal ions in biology, biochemistry, and medicine · 2025
    Article
  6. Article
  7. Article
  8. Article
  9. Psychiatric Symptoms in Wilson's Disease-Consequence ofInternational journal of molecular sciences · 2024
    Review
  10. Article
  11. Article
  12. Review
  13. Iron, Copper, and Selenium: Cancer's Thing for Redox Bling.Cold Spring Harbor perspectives in medicine · 2024
    Review
  14. Article
  15. Article
  16. Article
  17. Article
  18. Secretion and uptake of copper via a small copper carrier in blood fluid.Metallomics : integrated biometal science · 2022
    Article
  19. The Rat Genome Database (RGD) facilitates genomic and phenotypic data integration across multiple species for biomedical research.Mammalian genome : official journal of the International Mammalian Genome Society · 2022
    Article
  20. Wilson Disease: Update on Pathophysiology and Treatment.Frontiers in cell and developmental biology · 2022
    Review
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

3 authors at 1 institution in 1 country.

Emily ReedSheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, Sheffield, UK.
Svetlana LutsenkoDepartment of Physiology, John Hopkins Medical Institutes, Baltimore, Maryland, USA.
Oliver BandmannSheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, Sheffield, UK.ORCID 0000-0003-3149-0252
University of Sheffield · GB

Funding

Molecular Mechanisms of ATP-dependent Copper TransportersR01DK071865 · NIDDK · OREGON HEALTH & SCIENCE UNIVERSITY · PI SVETLANA LUTSENKO · 2005 to 2026
$7.9M
Integrative Analysis of Wilson's DiseaseR01GM101502 · NIGMS · JOHNS HOPKINS UNIVERSITY · PI LUTSENKO, SVETLANA · 2012 to 2021
$3.6M
NIDDK NIH HHS R01 DK071865NIGMS NIH HHS R01 GM101502
6 · The paper itself

Abstract

Wilson disease (WD) is an autosomal recessive disorder of copper metabolism manifesting with hepatic, neurological and psychiatric symptoms. The limitations of the currently available therapy for WD (particularly in the management of neuropsychiatric disease), together with our limited understanding of key aspects of this illness (e.g. neurological vs. hepatic presentation) justify the ongoing need to study WD in suitable animal models. Four animal models of WD have been established: the Long-Evans Cinnamon rat, the toxic-milk mouse, the Atp7b knockout mouse and the Labrador retriever. The existing models of WD all show good similarity to human hepatic WD and have been helpful in developing an improved understanding of the human disease. As mammals, the mouse, rat and canine models also benefit from high homology to the human genome. However, important differences exist between these mammalian models and human disease, particularly the absence of a convincing neurological phenotype. This review will first provide an overview of our current knowledge of the orthologous genes encoding ATP7B and the closely related ATP7A protein in C. elegans, Drosophila and zebrafish (Danio rerio) and then summarise key characteristics of rodent and larger mammalian models of ATP7B-deficiency.

Indexed as

Disease Models, AnimalHepatolenticular DegenerationAnimalsAnimals, Genetically ModifiedCopper-Transporting ATPasesHumansMutationATP7B protein, humanCopper-Transporting ATPasesAnimal modelsWilson disease

Identifiers

PMID29473169
PMCPMC6107386
OpenAlexW2792332276

What OpenQuestion holds

Textmetadata
LicenceTDM
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.