ArticleArteriosclerosis, thrombosis, and vascular biology2023
Lineage-Specific Induced Pluripotent Stem Cell-Derived Smooth Muscle Cell Modeling Predicts Integrin Alpha-V Antagonism Reduces Aortic Root Aneurysm Formation in Marfan Syndrome Mice.
Article in Arteriosclerosis, thrombosis, and vascular biology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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26 citing papers in PubMed, 33 citations in OpenAlex.
- The aorta in Marfan syndrome: from molecular mechanisms to mechanobiological dysfunction.Cardiovascular research · 2026Review
- Non-Contact Transspatial Regulatory Function of the Extracellular Matrix.Smart medicine · 2026Article
- Fibronectin-induced overactivation of αNature communications · 2026Article
- Focal adhesion proteins confer smooth muscle anoikis resistance and protection against aortic aneurysm and dissection.JCI insight · 2026Article
- FGF12 induces aberrant mechanosignaling in aortic smooth muscle cells during thoracic aortic aneurysm formation in Marfan syndrome mice.Experimental & molecular medicine · 2026Article
- Identification of PPIA, DPP4, and ITK as Potential Targets of Pterostilbene in Abdominal Aortic Aneurysm: A Network Pharmacology and Molecular Docking Study.Current medicinal chemistry · 2026Article
- Transplantation of iPSC-derived vascular wall-like tissue for improved embolization and reconstruction of aneurysm sac in endovascular aneurysm repair.Frontiers in bioengineering and biotechnology · 2026Article
- Double-Edged Signaling: FAK's Role in Aortic Dissection Progression.JACC. Basic to translational science · 2025Article
- Loeys-Dietz syndrome subtypes exhibit distinct clinical behavior and aortic cellular transcriptomic profiles.JTCVS open · 2025Article
- Integrin-mediated mTOR signaling drives TGF-β overactivity and myxomatous mitral valve degeneration in hypomorphic fibrillin-1 mice.The Journal of clinical investigation · 2025Article
- Induced Pluripotent (iPSC) and Mesenchymal (MSC) Stem Cells for In Vitro Disease Modeling and Regenerative Medicine.International journal of molecular sciences · 2025Review
- Chemokine (C-C Motif) Ligand 2 Expressing Adventitial Fibroblast Expansion During Loeys-Dietz Syndrome Aortic Aneurysm Formation.Arteriosclerosis, thrombosis, and vascular biology · 2025Article
- Origins of Aortic Coarctation: A Vascular Smooth Muscle Compartment Boundary Model.Journal of developmental biology · 2025Article
- Single-Cell Transcriptomics Identifies Selective Lineage-Specific Regulation of Genes in Aortic Smooth Muscle Cells in Mice.Arteriosclerosis, thrombosis, and vascular biology · 2025Article
- Stem cell-based therapies for treatment of abdominal aortic aneurysm: development, application, and future potential.npj biomedical innovations · 2025Review
- Therapeutic Opportunities of Marfan Syndrome: Current Perspectives.Drug design, development and therapy · 2025Review
- Identification of Disease-Relevant, Sex-Based Proteomic Differences in iPSC-Derived Vascular Smooth Muscle Cells.International journal of molecular sciences · 2024Article
- Transcriptional regulation of postnatal aortic development.Cells & development · 2024Article
- Use of iPSC-Derived Smooth Muscle Cells to Model Physiology and Pathology.Arteriosclerosis, thrombosis, and vascular biology · 2024Review
- Integrins as Drug Targets in Vascular and Related Diseases.International journal of drug discovery and pharmacology · 2024Article
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Authors and funding
13 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundThe role of increased smooth muscle cell (SMC) integrin αv signaling in Marfan syndrome (MFS) aortic aneurysm remains unclear. Herein, we examine the mechanism and potential efficacy of integrin αv blockade as a therapeutic strategy to reduce aneurysm progression in MFS.
methodsInduced pluripotent stem cells (iPSCs) were differentiated into aortic SMCs of the second heart field (SHF) and neural crest (NC) lineages, enabling in vitro modeling of MFS thoracic aortic aneurysms. The pathological role of integrin αv during aneurysm formation was confirmed by blockade of integrin αv with GLPG0187 in
resultsiPSC-derived MFS SHF SMCs overexpress integrin αv relative to MFS NC and healthy control SHF cells. Furthermore, integrin αv downstream targets (FAK [focal adhesion kinase]/Akt
conclusionsThe integrin αv-FAK-Akt
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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.