Evidence map›Paper›PMID 27727273›Full record

ArticlePLoS genetics2016

Zfp423 Regulates Sonic Hedgehog Signaling via Primary Cilium Function.

Chen-Jei Hong, Bruce A Hamilton

Open access · goldAbstract read
In one paragraph

Article in PLoS genetics, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.

0numbers the graph read from it
0cells of the map it votes in
22citing papers in PubMed
2.1field-weighted citation impact, top 11% 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

22 citing papers in PubMed, 32 citations in OpenAlex.

  1. Broadening horizons: Pathogenesis and therapeutics of renal ciliopathies.Journal of cell communication and signaling · 2026
    Review
  2. Article
  3. Article
  4. bioRxiv : the preprint server for biology · 2025
    Article
  5. Article
  6. Review
  7. Article
  8. Sonic Hedgehog Signaling in Cerebellar Development and Cancer.Frontiers in cell and developmental biology · 2022
    Review
  9. Ciliary control of adipocyte progenitor cell fate regulates energy storage.Frontiers in cell and developmental biology · 2022
    Review
  10. Article
  11. Article
  12. Renal Ciliopathies: Sorting Out Therapeutic Approaches for Nephronophthisis.Frontiers in cell and developmental biology · 2021
    Review
  13. Article
  14. Article
  15. Article
  16. Article
  17. Review
  18. Article
  19. Article
  20. 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

2 authors at 1 institution in 1 country.

Chen-Jei HongDepartment of Cellular & Molecular Medicine, University of California, San Diego School of Medicine, La Jolla, California, United States of America.ORCID http://orcid.org/0000-0003-0142-5919
Bruce A HamiltonDepartment of Cellular & Molecular Medicine, University of California, San Diego School of Medicine, La Jolla, California, United States of America.
University of California San Diego · US

Funding

Genetic Analysis of Neural Stem CellsR01NS060109 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI HAMILTON, BRUCE A · 2008 to 2012
$1.7M
Genetic Mechanisms in Cerebellum MalformationsR01NS054871 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI HAMILTON, BRUCE A · 2007 to 2011
$1.6M
Synthetic Lethal Modifier of a New Ciliopathy GeneR01GM102958 · NIGMS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI HAMILTON, BRUCE A · 2012 to 2015
$1.2M
NIGMS NIH HHS R01 GM102958NINDS NIH HHS R01 NS054871NINDS NIH HHS R01 NS060109
6 · The paper itself

Abstract

Zfp423 encodes a 30-zinc finger transcription factor that intersects several canonical signaling pathways. Zfp423 mutations result in ciliopathy-related phenotypes, including agenesis of the cerebellar vermis in mice and Joubert syndrome (JBTS19) and nephronophthisis (NPHP14) in humans. Unlike most ciliopathy genes, Zfp423 encodes a nuclear protein and its developmental expression is complex, leading to alternative proposals for cellular mechanisms. Here we show that Zfp423 is expressed by cerebellar granule cell precursors, that loss of Zfp423 in these precursors leads to cell-intrinsic reduction in proliferation, loss of response to Shh, and primary cilia abnormalities that include diminished frequency of both Smoothened and IFT88 localization. Loss of Zfp423 alters expression of several genes encoding key cilium components, including increased expression of Tulp3. Tulp3 is a direct binding target of Zfp423 and reducing the overexpression of Tulp3 in Zfp423-deficient cells suppresses Smoothened translocation defects. These results define Zfp423 deficiency as a bona fide ciliopathy, acting upstream of Shh signaling, and indicate a mechanism intrinsic to granule cell precursors for the resulting cerebellar hypoplasia.

Indexed as

Abnormalities, MultipleAnimalsCerebellumCiliaCiliopathiesDevelopmental DisabilitiesDNA-Binding ProteinsEmbryo, MammalianEye AbnormalitiesGene Expression Regulation, DevelopmentalHedgehog ProteinsHumansIntercellular Signaling Peptides and ProteinsIntracellular Signaling Peptides and ProteinsKidney Diseases, CysticMiceDNA-Binding ProteinsEbfaz protein, mouseHedgehog ProteinsIntercellular Signaling Peptides and ProteinsIntracellular Signaling Peptides and ProteinsProteinsTranscription FactorsTulp3 protein, mouse

Identifiers

PMID27727273
PMCPMC5065120
OpenAlexW2949915089

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.