ArticleThe Journal of clinical investigation2003
Developmental adaptation of the mouse cardiovascular system to elastin haploinsufficiency.
Article in The Journal of clinical investigation, 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 137 papers.
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Who cites it
137 citing papers in PubMed, 247 citations in OpenAlex.
- Minoxidil versus placebo in the treatment of arterial wall hypertrophy in children with Williams Beuren Syndrome: a randomized controlled trial.BMC pediatrics · 2019Trial
- Novel C-terminal frameshift mutation of ELN causes non-stenotic high-renin hypertension and systemic arterial dysplasia: implications for preventing aortic catastrophes.Hypertension research : official journal of the Japanese Society of Hypertension · 2026Article
- Mechanics of Balloon and Stent Angioplasty in Congenital Heart Interventions.Pediatric cardiology · 2026Review
- Neuraminidase1 Activity Contributes to Vasopressin Receptor-mediated Augmentation of Water and Electrolyte Retention by the Kidney inbioRxiv : the preprint server for biology · 2026Article
- Ovarian hormones moderate systolic hypertension in femaleAmerican journal of physiology. Renal physiology · 2026Article
- Elastin Regulation of Vasoreactivity in Resistance Arteries.Hypertension (Dallas, Tex. : 1979) · 2025Article
- Ovarian Hormones Moderate Systolic Hypertension in FemalebioRxiv : the preprint server for biology · 2025Article
- A Del(5PNAS nexus · 2025Article
- Unraveling Elastic Fiber-Derived Signaling in Arterial Aging and Related Arterial Diseases.Biomolecules · 2025Review
- Clinical Care for Cardiovascular Disease in Patients With Williams-Beuren Syndrome.Journal of the American Heart Association · 2024Review
- The Pathogenesis and Impact of Arterial Stiffening in Hypertension: The 2023 John H. Laragh Research Award.American journal of hypertension · 2024Article
- Constitutive Modeling of Mouse Arteries Suggests Changes in Directional Coupling and Extracellular Matrix Remodeling That Depend on Artery Type, Age, Sex, and Elastin Amounts.Journal of biomechanical engineering · 2024Article
- Novel mutation in ELN gene causes cardiac abnormalities and inguinal hernia: case report.BMC pediatrics · 2023Article
- Ophthalmic characteristics and retinal vasculature changes in Williams syndrome, and its association with systemic diseases.Eye (London, England) · 2023Article
- Changes in transmural mass transport correlate with ascending thoracic aortic aneurysm diameter in a fibulin-4 E57K knockin mouse model.American journal of physiology. Heart and circulatory physiology · 2023Article
- Characterization of the ZebrafishCells · 2023Article
- Pentagalloyl Glucose (PGG) Prevents and Restores Mechanical Changes Caused by Elastic Fiber Fragmentation in the Mouse Ascending Aorta.Annals of biomedical engineering · 2023Article
- Genetics and mechanisms of thoracic aortic disease.Nature reviews. Cardiology · 2023Review
- A new mouse model of elastin haploinsufficiency highlights the importance of elastin to vascular development and blood pressure regulation.Matrix biology : journal of the International Society for Matrix Biology · 2023Article
- Physiological Impact of a Synthetic Elastic Protein in Arterial Diseases Related to Alterations of Elastic Fibers: Effect on the Aorta of Elastin-Haploinsufficient Male and Female Mice.International journal of molecular sciences · 2022Article
77 more citing papers are in PubMed but not listed here.
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13 authors at 5 institutions in 2 countries.
Funding
Abstract
Supravalvular aortic stenosis is an autosomal-dominant disease of elastin (Eln) insufficiency caused by loss-of-function mutations or gene deletion. Recently, we have modeled this disease in mice (Eln+/-) and found that Eln haploinsufficiency results in unexpected changes in cardiovascular hemodynamics and arterial wall structure. Eln+/- animals were found to be stably hypertensive from birth, with a mean arterial pressure 25-30 mmHg higher than their wild-type counterparts. The animals have only moderate cardiac hypertrophy and live a normal life span with no overt signs of degenerative vascular disease. Examination of arterial mechanical properties showed that the inner diameters of Eln+/- arteries were generally smaller than wild-type arteries at any given intravascular pressure. Because the Eln+/- mouse is hypertensive, however, the effective arterial working diameter is comparable to that of the normotensive wild-type animal. Physiological studies indicate a role for the renin-angiotensin system in maintaining the hypertensive state. The association of hypertension with elastin haploinsufficiency in humans and mice strongly suggests that elastin and other proteins of the elastic fiber should be considered as causal genes for essential hypertension.
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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.