Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.
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Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes Afford New Opportunities in Inherited Cardiovascular Disease ModelingReduction of NADPH-oxidase activity ameliorates the cardiovascular phenotype in a mouse model of Williams-Beuren SyndromeInduced chromosome deletion in a Williams-Beuren syndrome mouse model causes cardiovascular abnormalities.Peripheral pulmonary artery stenosis as a cause of pulmonary hypertension in adultsSystematic identification and characterization of chicken (Gallus gallus) ncRNAsThe Drosophila agnostic Locus: Involvement in the Formation of Cognitive Defects in Williams SyndromeThe Williams-Beuren Syndrome-a window into genetic variants leading to the development of cardiovascular disease.Williams syndrome as a model for elucidation of the pathway genes - the brain - cognitive functions: genetics and epigeneticsGenetic modifiers of cardiovascular phenotype caused by elastin haploinsufficiency act by extrinsic noncomplementation.High prevalence of cardiovascular risk factors in children and adolescents with Williams-Beuren syndromeInhibition of microRNA-29 enhances elastin levels in cells haploinsufficient for elastin and in bioengineered vessels--brief report.Elastin Insufficiency Predisposes Mice to Impaired Glucose Metabolism.Integrin β3 inhibition is a therapeutic strategy for supravalvular aortic stenosis.Alternative splicing and tissue-specific elastin misassembly act as biological modifiers of human elastin gene frameshift mutations associated with dominant cutis laxa.Elastin in large artery stiffness and hypertension.Chronic antihypertensive treatment improves pulse pressure but not large artery mechanics in a mouse model of congenital vascular stiffnessEngineered zinc-finger proteins can compensate genetic haploinsufficiency by transcriptional activation of the wild-type allele: application to Willams-Beuren syndrome and supravalvular aortic stenosisTwenty patients including 7 probands with autosomal dominant cutis laxa confirm clinical and molecular homogeneity.Mechanisms of emphysema in autosomal dominant cutis laxa.Elastin insufficiency predisposes to elevated pulmonary circulatory pressures through changes in elastic artery structureWilliams syndrome predisposes to vascular stiffness modified by antihypertensive use and copy number changes in NCF1Altered reactivity of resistance vasculature contributes to hypertension in elastin insufficiency.Williams-Beuren syndrome: computed tomography imaging review.Supravalvular aortic stenosis in William's syndrome.Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review.Stiff Substrates Increase Inflammation-Induced Endothelial Monolayer Tension and Permeability.Description of common musculoskeletal findings in Williams Syndrome and implications for therapies.What is the association of acute renal failure, angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker in a young patient?Intestinal epithelial cell-derived integrin αβ6 plays an important role in the induction of regulatory T cells and inhibits an antigen-specific Th2 response.Modeling supravalvular aortic stenosis syndrome with human induced pluripotent stem cells.Williams syndrome and Ebstein's anomaly: A rare association.Loss of Smooth Muscle α-Actin Leads to NF-κB-Dependent Increased Sensitivity to Angiotensin II in Smooth Muscle Cells and Aortic Enlargement.Parsing aortic aneurysms: more surprises.Restoring elastin with microRNA-29.Vascular Smooth Muscle Cells and Arterial Stiffening: Relevance in Development, Aging, and Disease.mTOR (Mechanistic Target of Rapamycin) Inhibition Decreases Mechanosignaling, Collagen Accumulation, and Stiffening of the Thoracic Aorta in Elastin-Deficient Mice.Deficient Circumferential Growth Is the Primary Determinant of Aortic Obstruction Attributable to Partial Elastin Deficiency.Heterozygous deletion of the Williams-Beuren syndrome critical interval in mice recapitulates most features of the human disorder.Using In Vivo and Tissue and Cell Explant Approaches to Study the Morphogenesis and Pathogenesis of the Embryonic and Perinatal Aorta.Minoxidil improves vascular compliance, restores cerebral blood flow and alters extracellular matrix gene expression in a model of chronic vascular stiffness.
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Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
@pt
bilimsel makale
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scientific article published on May 2008
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.
@en
Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.
@nl
type
label
Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.
@en
Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.
@nl
prefLabel
Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.
@en
Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.
@nl
P2860
P356
P1476
Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome
@en
P2093
Barbara R Pober
Mark Johnson
P2860
P304
P356
10.1172/JCI35309
P407
P50
P577
2008-05-01T00:00:00Z