ArticleArteriosclerosis, thrombosis, and vascular biology2017
Deficient Circumferential Growth Is the Primary Determinant of Aortic Obstruction Attributable to Partial Elastin Deficiency.
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Article in Arteriosclerosis, thrombosis, and vascular biology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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32citing papers in PubMed
3.2field-weighted citation impact, top 9% of its field
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32 citing papers in PubMed, 50 citations in OpenAlex.
- Sphingosine kinase 1 is integral for elastin deficiency-induced arterial hypermuscularization.Nature cardiovascular research · 2026Article
- Long Segment Midaortic Stenosis in Williams Syndrome: Report of a Very Rare Presentation.Medical journal of the Islamic Republic of Iran · 2025Article
- Presenilin-1 in smooth muscle cells facilitates hypermuscularization in elastin aortopathy.iScience · 2024Article
- Adult William's Syndrome: The Cause of an Unusual Vasculopathy and Biliary Abnormalities.Cureus · 2023Article
- Ophthalmic characteristics and retinal vasculature changes in Williams syndrome, and its association with systemic diseases.Eye (London, England) · 2023Article
- SCAP contributes to embryonic angiogenesis by negatively regulating KISS-1 expression in mice.Cell death & disease · 2023Article
- Thirty-Year Survival After Cardiac Surgery in Children With Williams-Beuren Syndrome (from the Pediatric Cardiac Care Consortium Study).The American journal of cardiology · 2023Article
- G2Φnet: Relating genotype and biomechanical phenotype of tissues with deep learning.PLoS computational biology · 2022Article
- Compromised Cardiopulmonary Function in Fibulin-5 Deficient Mice.Journal of biomechanical engineering · 2022Article
- Elastin, arterial mechanics, and stenosis.American journal of physiology. Cell physiology · 2022Review
- JAGGED1/NOTCH3 activation promotes aortic hypermuscularization and stenosis in elastin deficiency.The Journal of clinical investigation · 2022Article
- Elastin Insufficiency Confers Proximal and Distal Pulmonary Vasculopathy in Mice, Partially Remedied by the KFrontiers in cardiovascular medicine · 2022Article
- Pulmonary arteries of Williams syndrome patients exhibit altered serotonin metabolism genes and degenerated medial layer architecture.Pediatric research · 2021Article
- Williams syndrome.Nature reviews. Disease primers · 2021Review
- Authentication of In Situ Measurements for Thoracic Aortic Aneurysms in Mice.Arteriosclerosis, thrombosis, and vascular biology · 2021Article
- Loss of Transforming Growth Factor Beta Signaling in Aortic Smooth Muscle Cells Causes Endothelial Dysfunction and Aortic Hypercontractility.Arteriosclerosis, thrombosis, and vascular biology · 2021Article
- Hypertension in Children and Adolescents with Turner Syndrome (TS), Neurofibromatosis 1 (NF1), and Williams Syndrome (WS).Current hypertension reports · 2021Review
- Mechanics-driven mechanobiological mechanisms of arterial tortuosity.Science advances · 2020Article
- Vascular elastic fiber heterogeneity in health and disease.Current opinion in hematology · 2020Review
- Stenosis coexists with compromised α1-adrenergic contractions in the ascending aorta of a mouse model of Williams-Beuren syndrome.Scientific reports · 2020Article
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Authors and funding
11 authors at 5 institutions in 2 countries.
Yang JiaoFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Guangxin LiFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Arina KornevaFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Alexander W CaulkFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Lingfeng QinFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Matthew R BersiFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Qingle LiFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Wei LiFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Robert P MechamFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
Jay D HumphreyFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.).
George TellidesFrom the Department of Surgery, Yale University School of Medicine, New Haven, CT (Y.J., G.L., L.Q., Q.L., W.L., G.T.); Department of Vascular Surgery, Peking University People's Hospital, Beijing, People's Republic of China (Y.J., Q.L., W.L.); Department of Vascular Surgery, The First Hospital of China Medical University, Shenyang, People's Republic of China (G.L.); Department of Biomedical Engineering, Yale University, New Haven, CT (A.K., A.W.C., M.R.B., J.D.H.); Department of Cell Biology, Washington University School of Medicine, St Louis, MO (R.P.M.); Interdepartmental Program in Vascular Biology and Therapeutics, Yale University School of Medicine (J.D.H., G.T.); and Veterans Affairs Connecticut Healthcare System, West Haven (G.T.). george.tellides@yale.edu.
Washington University in St. Louis · USFirst Hospital of China Medical University · CNChina Medical University · CNPeking University People's Hospital · CNYale University · US
Funding
Yale Clinical and Translational Science Award (U Component)UL1TR001863 · NCATS · YALE UNIVERSITY · PI John H. Krystal, LUCILA OHNO-MACHADO · 2016 to 2026
$102.9MASSEMBLY OF LUNG ELASTINR37HL053325 · NHLBI · WASHINGTON UNIVERSITY · PI MECHAM, ROBERT P. · 2000 to 2009
$4.1MBiomechanical Simulation of Evolving Aortic Aneurysms for Designing InterventionR01HL086418 · NHLBI · YALE UNIVERSITY · PI HUMPHREY, JAY D. · 2008 to 2017
$3.8MASSEMBLY OF LUNG ELASTINR01HL053325 · NHLBI · WASHINGTON UNIVERSITY · PI MECHAM, ROBERT P. · 1995 to 2019
$3.6MVESSEL STIFFENING, HYPERTENSION, AND VASCULAR EXTRACELLULAR MATRIXR01HL105314 · NHLBI · WASHINGTON UNIVERSITY · PI MECHAM, ROBERT P., WAGENSEIL, JESSICA · 2010 to 2018
$3.2MMechanisms Underlying The Progression of Large Artery Stiffness in HypertensionR01HL105297 · NHLBI · YALE UNIVERSITY · PI FIGUEROA, CARLOS ALBERTO, HUMPHREY, JAY D. · 2010 to 2019
$3.0MNCATS NIH HHS UL1 TR001863NHLBI NIH HHS R01 HL053325NHLBI NIH HHS R01 HL086418NHLBI NIH HHS R01 HL105297NHLBI NIH HHS R01 HL105314NHLBI NIH HHS R37 HL053325
Abstract
objectiveWilliams syndrome is characterized by obstructive aortopathy attributable to heterozygous loss of APPROACH AND
resultsWe quantified determinants of luminal stenosis in thoracic aortas of
conclusionsDeficient circumferential growth is the predominant mechanism for moderate obstructive aortic disease resulting from partial elastin deficiency. Our findings suggest that diverse aortic manifestations in Williams syndrome result from graded elastin content, and SMC hyperplasia causing medial expansion requires additional elastin loss superimposed on
Indexed as
AdultAnimalsAorta, ThoracicAortic DiseasesCell ProliferationCells, CulturedCollagenConstriction, PathologicDisease Models, AnimalElastinFibrosisGenetic Predisposition to DiseaseHumansHyperplasiaMaleMice, Inbred C57BLCollagenElastinaortacollagenelastinsmooth muscle cellsvascular remodeling
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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.