Evidence map›Paper›PMID 23868979›Full record

ReviewJournal of cell science2013

Modelling of human Wiskott-Aldrich syndrome protein mutants in zebrafish larvae using in vivo live imaging.

Rebecca A Jones, Yi Feng, Austen J Worth, Adrian J Thrasher, Siobhan O Burns, Paul Martin

Open access · hybridAbstract readReview
PubMed Full text at PMC Publisher
In one paragraph

Review in Journal of cell science, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed
1.0field-weighted citation impact, top 23% 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

17 citing papers in PubMed, 30 citations in OpenAlex.

  1. Article
  2. Review
  3. Review
  4. Review
  5. Article
  6. Article
  7. Article
  8. Zebrafish Cancer Predisposition Models.Frontiers in cell and developmental biology · 2021
    Review
  9. Review
  10. Article
  11. Review
  12. Modeling hematopoietic disorders in zebrafish.Disease models & mechanisms · 2019
    Review
  13. Review
  14. Article
  15. Article
  16. Review
  17. Article
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

6 authors at 4 institutions in 1 country.

Rebecca A JonesSchool of Biochemistry, Faculty of Medical and Veterinary Sciences, University of Bristol, University Walk, Bristol, BS8 1TD, UK.
Yi Feng
Austen J Worth
Adrian J Thrasher
Siobhan O Burns
Paul Martin
Great Ormond Street Hospital · GBUniversity of Bristol · GBQueen's Medical Centre · GBThe Royal Free Hospital · GB

Funding

Medical Research CouncilWellcome Trust 072725/Z/03/ZWellcome Trust 077988/Z/05/ZWellcome Trust 090233/Z/09/ZWellcome Trust 097791Wellcome Trust 097791MAWellcome Trust 100104Wellcome Trust 100104/Z/12/Z
6 · The paper itself

Abstract

Wiskott-Aldrich syndrome (WAS) and X-linked neutropenia (XLN) are immunodeficiencies in which the function of several haematopoietic cell lineages is perturbed as a result of mutations in the actin regulator WASp. From in vitro cell biology experiments, and biochemical and structural approaches, we know much about the functional domains of WASp and how WASp might regulate the dynamic actin cytoskeleton downstream of activators such as Cdc42, but in vivo experiments are much more challenging. In patients, there is a correlation between clinical disease and genotype, with severe reductions in WASp expression or function associating with complex multilineage immunodeficiency, whereas specific mutations that cause constitutive activation of WASp result in congenital neutropenia. Here, we take advantage of the genetic tractability and translucency of zebrafish larvae to first characterise how a null mutant in zfWASp influences the behaviour of neutrophils and macrophages in response to tissue damage and to clearance of infections. We then use this mutant background to study how leukocyte lineage-specific transgenic replacement with human WASp variants (including normal wild type and point mutations that either fail to bind Cdc42 or cannot be phosphorylated, and a constitutively active mutant equivalent to that seen in XLN patients) alter the capacity for generation of neutrophils, their chemotactic response to wounds and the phagocytic clearance capacity of macrophages. This model provides a unique insight into WASp-related immunodeficiency at both a cellular and whole organism level.

Indexed as

AnimalsCongenital Bone Marrow Failure SyndromesDisease Models, AnimalHumansMacrophagesNeutropeniaWiskott-Aldrich SyndromeZebrafishDisease modelImmune deficiencyLive imagingMacrophagesNeutrophilsWASpZebrafish

Identifiers

PMID23868979
PMCPMC3772384
OpenAlexW2083724896

What OpenQuestion holds

Textmetadata
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.