Trial reportThe Journal of clinical investigation2020
Impaired angiogenesis and extracellular matrix metabolism in autosomal-dominant hyper-IgE syndrome.
Trial report in The Journal of clinical investigation, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04864886 (Metabolic Profiling of Immune Responses in Immune-Mediated Diseases), which is not on this map. Cited by 21 papers.
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.
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.
Metabolic Profiling of Immune Responses in Immune-Mediated Diseases
Who cites it
21 citing papers in PubMed, 25 citations in OpenAlex.
- Differential responses to folic acid in an established keloid fibroblast cell line are mediated by JAK1/2 and STAT3.PloS one · 2021Trial
- Clinical features, genetics, treatment, and long-term outcomes of STAT3 hyper-IgE syndrome: Single-center cohort analysis.The Journal of allergy and clinical immunology · 2026Observational
- Article
- Insight into the research frontier of hypoxia in skin health and diseases: a bibliometric analysis.Annals of medicine and surgery (2012) · 2025Review
- Successful coil occlusion of intracranial aneurysm in a child with STAT3 hyper IgE syndrome.Journal of human immunity · 2025Article
- Serum periostin as a novel biomarker and therapeutic target in coronary heart disease.Frontiers in cardiovascular medicine · 2025Article
- Defective Endothelial Glutaminolysis Contributes to Impaired Angiogenesis and Poor Ischemic Tissue Repair in Diabetes.Research (Washington, D.C.) · 2025Article
- Case Report: Biliary hemorrhage by intrahepatic pseudoaneurysm and asymptomatic right coronary artery pseudoaneurysm in a patient with STAT3 hyper IgE syndrome.Frontiers in immunology · 2025Article
- Tail Fin Regeneration in Zebrafish: The Role of Non-canonical Crosstalk Between STAT3 and Vitamin D Pathway.International journal of biological sciences · 2025Article
- Self-assembly of PEG-PPS polymers and LL-37 peptide nanomicelles improves the oxidative microenvironment and promotes angiogenesis to facilitate chronic wound healing.Bioengineering & translational medicine · 2024Article
- Identification of biomarkers related to angiogenesis in myocardial ischemia-reperfusion injury and prediction of potential drugs.PloS one · 2024Article
- STAT3 Deficiency Alters the Macrophage Activation Pattern and Enhances Matrix Metalloproteinase 9 Expression during Staphylococcal Pneumonia.Journal of immunology (Baltimore, Md. : 1950) · 2024Article
- Recurrent Breast Abscesses in a Female with Autosomal Dominant Hyper-IgE Syndrome.Journal of clinical immunology · 2022Article
- Development of vascular disease models to explore disease causation and pathomechanisms of rare vascular diseases.Seminars in immunopathology · 2022Review
- STAT3 is critical for skeletal development and bone homeostasis by regulating osteogenesis.Nature communications · 2021Article
- STAT3 Hyper-IgE Syndrome-an Update and Unanswered Questions.Journal of clinical immunology · 2021Review
- A comprehensive and universal approach for embryo testing in patients with different genetic disorders.Clinical and translational medicine · 2021Article
- An Unusual Pattern of Premature Cervical Spine Degeneration in STAT3-LOF.Journal of clinical immunology · 2021Article
- The Child with Elevated IgE and Infection Susceptibility.Current allergy and asthma reports · 2020Review
- Tumor Necrosis Factor Receptors: Pleiotropic Signaling Complexes and Their Differential Effects.Frontiers in immunology · 2020Review
Corrections and comments
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Authors and funding
27 authors at 7 institutions in 3 countries.
Funding
Abstract
There are more than 7000 described rare diseases, most lacking specific treatment. Autosomal-dominant hyper-IgE syndrome (AD-HIES, also known as Job's syndrome) is caused by mutations in STAT3. These patients present with immunodeficiency accompanied by severe nonimmunological features, including skeletal, connective tissue, and vascular abnormalities, poor postinfection lung healing, and subsequent pulmonary failure. No specific therapies are available for these abnormalities. Here, we investigated underlying mechanisms in order to identify therapeutic targets. Histological analysis of skin wounds demonstrated delayed granulation tissue formation and vascularization during skin-wound healing in AD-HIES patients. Global gene expression analysis in AD-HIES patient skin fibroblasts identified deficiencies in a STAT3-controlled transcriptional network regulating extracellular matrix (ECM) remodeling and angiogenesis, with hypoxia-inducible factor 1α (HIF-1α) being a major contributor. Consistent with this, histological analysis of skin wounds and coronary arteries from AD-HIES patients showed decreased HIF-1α expression and revealed abnormal organization of the ECM and altered formation of the coronary vasa vasorum. Disease modeling using cell culture and mouse models of angiogenesis and wound healing confirmed these predicted deficiencies and demonstrated therapeutic benefit of HIF-1α-stabilizing drugs. The study provides mechanistic insights into AD-HIES pathophysiology and suggests potential treatment options for this rare disease.
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What OpenQuestion holds
Registered trials
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.