Evidence map›Paper›PMID 31469182›Full record

ArticleHepatology (Baltimore, Md.)2020

Sox9 Is a Modifier of the Liver Disease Severity in a Mouse Model of Alagille Syndrome.

Joshua M Adams, Kari A Huppert, Eumenia C Castro, Mario F Lopez, Nima Niknejad, Sanjay Subramanian, Neda Zarrin-Khameh, Milton J Finegold, Stacey S Huppert, Hamed Jafar-Nejad

Open access · greenAbstract read
In one paragraph

Article in Hepatology (Baltimore, Md.), 2020. 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
3.5field-weighted citation impact, top 7% 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, 35 citations in OpenAlex.

  1. Article
  2. Phenotypic Divergence of JAG1- and NOTCH2-Associated Alagille Syndrome & Disease-Specific NOTCH2 Variant Classification Guidelines.Liver international : official journal of the International Association for the Study of the Liver · 2025
    Article
  3. Article
  4. Review
  5. Article
  6. Article
  7. Article
  8. Review
  9. Review
  10. Review
  11. Review
  12. Article
  13. Review
  14. Review
  15. Regenerative failure of intrahepatic biliary cells in Alagille syndrome rescued by elevated Jagged/Notch/Sox9 signaling.Proceedings of the National Academy of Sciences of the United States of America · 2022
    Article
  16. Notch signaling pathway: architecture, disease, and therapeutics.Signal transduction and targeted therapy · 2022
    Review
  17. Role of Immune Cells in Biliary Repair.Frontiers in immunology · 2022
    Review
  18. Article
  19. Tissue engineering. Part C, Methods · 2021
    Review
  20. 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

10 authors at 2 institutions in 1 country.

Joshua M AdamsProgram in Developmental Biology, Baylor College of Medicine, Houston, TX.ORCID 0000-0002-9556-9499
Kari A HuppertDivision of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Eumenia C CastroDepartment of Pathology & Immunology, Baylor College of Medicine, Houston, TX.
Mario F LopezDepartment of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX.
Nima NiknejadDepartment of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX.
Sanjay SubramanianDivision of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Neda Zarrin-KhamehDepartment of Pathology & Immunology, Baylor College of Medicine, Houston, TX.
Milton J FinegoldDepartment of Pathology & Immunology, Baylor College of Medicine, Houston, TX.
Stacey S HuppertDivision of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Hamed Jafar-NejadProgram in Developmental Biology, Baylor College of Medicine, Houston, TX.
Baylor College of Medicine · USCincinnati Children's Hospital Medical Center · US

Funding

Tissue Analysis & Molecular Imaging CoreP30DK056338 · NIDDK · BAYLOR COLLEGE OF MEDICINE · PI Geoffrey A Preidis · 2001 to 2026
$28.3M
Stem Cell/Organoid and Genome Editing CoreP30DK078392 · NIDDK · CINCINNATI CHILDRENS HOSP MED CTR · PI Alexander Miethke · 2007 to 2026
$24.4M
Preclinical and Clincial OutcomesP50HD103555 · NICHD · BAYLOR COLLEGE OF MEDICINE · PI MIRJANA MALETIC-SAVATIC · 2020 to 2026
$9.9M
Steps towards a paternal gene activation therapy for Angelman syndromeU54HD083092 · NICHD · BAYLOR COLLEGE OF MEDICINE · PI NELSON, DAVID LOREN, SAMACO, RODNEY C · 2014 to 2019
$7.8M
Roles of Glycosylation and Deglycosylation During Animal DevelopmentR35GM130317 · NIGMS · BAYLOR COLLEGE OF MEDICINE · PI Hamed Jafar-Nejad · 2019 to 2026
$3.7M
Fine-Tuning the Notch Signaling Pathway via O-GlucosylationR01GM084135 · NIGMS · UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON · PI JAFAR-NEJAD, HAMED · 2008 to 2017
$2.6M
Building a functional biliary system from hepatocytesR01DK107553 · NIDDK · CINCINNATI CHILDRENS HOSP MED CTR · PI HUPPERT, STACEY S, WILLENBRING, HOLGER · 2016 to 2020
$2.4M
Negative regulation of Jagged1 by glycosylation: towards a mechanism-based therapy for Alagille syndromeR01DK109982 · NIDDK · BAYLOR COLLEGE OF MEDICINE · PI JAFAR-NEJAD, HAMED · 2016 to 2019
$1.6M
Molecular requirements for proliferation of fetal and adult liver progenitorsR01DK078640 · NIDDK · VANDERBILT UNIVERSITY · PI HUPPERT, STACEY S · 2008 to 2012
$1.5M
NICHD NIH HHS P50 HD103555NICHD NIH HHS U54 HD083092NIDDK NIH HHS DK078392NIDDK NIH HHS DK078640NIDDK NIH HHS DK107553NIDDK NIH HHS DK109982NIDDK NIH HHS DK56338NIDDK NIH HHS P30 DK056338NIDDK NIH HHS P30 DK078392NIDDK NIH HHS R01 DK078640NIDDK NIH HHS R01 DK107553NIDDK NIH HHS R01 DK109982NIGMS NIH HHS GM084135NIGMS NIH HHS R01 GM084135NIGMS NIH HHS R35 GM130317
6 · The paper itself

Abstract

BACKGROUND AND

aimsAlagille syndrome (ALGS) is a multisystem developmental disorder characterized by bile duct (BD) paucity, caused primarily by haploinsufficiency of the Notch ligand jagged1. The course of the liver disease is highly variable in ALGS. However, the genetic basis for ALGS phenotypic variability is unknown. Previous studies have reported decreased expression of the transcription factor SOX9 (sex determining region Y-box 9) in late embryonic and neonatal livers of Jag1-deficient mice. Here, we investigated the effects of altering the Sox9 gene dosage on the severity of liver disease in an ALGS mouse model. APPROACH AND

resultsConditional removal of one copy of Sox9 in Jag1

conclusionsOur results establish Sox9 as a dosage-sensitive modifier of Jag1

Indexed as

Alagille SyndromeAnimalsBile DuctsCell TransdifferentiationChildChild, PreschoolDisease Models, AnimalHepatocytesHeterozygoteHumansInfantJagged-1 ProteinLiverMiceMice, Inbred C57BLReceptors, NotchJag1 protein, mouseJagged-1 ProteinReceptors, NotchSox9 protein, mouseSOX9 Transcription Factor

Identifiers

PMID31469182
PMCPMC7048647
OpenAlexW2970687891

What OpenQuestion holds

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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.