Novel arterial pathology in mice and humans hemizygous for elastin.
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Submicroscopic deletion in patients with Williams-Beuren syndrome influences expression levels of the nonhemizygous flanking genesNeuraminidase-1, a subunit of the cell surface elastin receptor, desialylates and functionally inactivates adjacent receptors interacting with the mitogenic growth factors PDGF-BB and IGF-2Of mice and men: molecular genetics of congenital heart diseaseReduction of NADPH-oxidase activity ameliorates the cardiovascular phenotype in a mouse model of Williams-Beuren SyndromeCharacterization of Microfibrillar-associated Protein 4 (MFAP4) as a Tropoelastin- and Fibrillin-binding Protein Involved in Elastic Fiber FormationToward a multi-scale computational model of arterial adaptation in hypertension: verification of a multi-cell agent based model.Evidence for heterozygous abnormalities of the elastin gene (ELN) affecting the quantity of vocal fold elastic fibers: a pilot study.Induced chromosome deletion in a Williams-Beuren syndrome mouse model causes cardiovascular abnormalities.Peripheral pulmonary artery stenosis as a cause of pulmonary hypertension in adultsDiscrete contributions of elastic fiber components to arterial development and mechanical compliance.Fibulin-4 is essential for maintaining arterial wall integrity in conduit but not muscular arteries.Matrix modulation of compensatory lung regrowth and progenitor cell proliferation in mice.Marfan syndrome: new clues to genotype-phenotype correlations.Altered vascular remodeling in fibulin-5-deficient mice reveals a role of fibulin-5 in smooth muscle cell proliferation and migration.Reduced fear and aggression and altered serotonin metabolism in Gtf2ird1-targeted mice.Williams (Williams Beuren) syndrome: a distinct neurobehavioral disorder.Rearrangements of the Williams-Beuren syndrome locus: molecular basis and implications for speech and language development.Elastic and collagenous networks in vascular diseasesMechanical property characterization of electrospun recombinant human tropoelastin for vascular graft biomaterials.The extracellular matrix can regulate vascular cell migration, proliferation, and survival: relationships to vascular disease.Increased expression of urokinase during atherosclerotic lesion development causes arterial constriction and lumen loss, and accelerates lesion growthThe genetic basis of aortic aneurysmThe importance of elastin to aortic development in mice.Congenital supravalvar aortic stenosis: a simple lesion?Williams syndrome: from genotype through to the cognitive phenotype.Molecular determinants of left and right outflow tract obstruction.Vascular wall extracellular matrix proteins and vascular diseasesHemizygosity at the NCF1 gene in patients with Williams-Beuren syndrome decreases their risk of hypertension.Fibrillins 1 and 2 perform partially overlapping functions during aortic development.Fibrillin-1 genetic deficiency leads to pathological ageing of arteries in mice.Decreased aortic diameter and compliance precedes blood pressure increases in postnatal development of elastin-insufficient mice.Mechanical factors direct mouse aortic remodelling during early maturation.Lung matrix and vascular remodeling in mechanically ventilated elastin haploinsufficient newborn mice.Williams-Beuren syndrome: a challenge for genotype-phenotype correlations.Cardiovascular manifestations in 75 patients with Williams syndrome.Genetic modifiers of cardiovascular phenotype caused by elastin haploinsufficiency act by extrinsic noncomplementation.Maladaptive matrix remodeling and regional biomechanical dysfunction in a mouse model of aortic valve disease.Genetic approaches to disease and regenerationAltered bladder function in elastin-deficient mice at baseline and in response to partial bladder outlet obstruction.Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology.
P2860
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P2860
Novel arterial pathology in mice and humans hemizygous for elastin.
description
1998 nî lūn-bûn
@nan
1998年の論文
@ja
1998年学术文章
@wuu
1998年学术文章
@zh-cn
1998年学术文章
@zh-hans
1998年学术文章
@zh-my
1998年学术文章
@zh-sg
1998年學術文章
@yue
1998年學術文章
@zh
1998年學術文章
@zh-hant
name
Novel arterial pathology in mice and humans hemizygous for elastin.
@en
Novel arterial pathology in mice and humans hemizygous for elastin.
@nl
type
label
Novel arterial pathology in mice and humans hemizygous for elastin.
@en
Novel arterial pathology in mice and humans hemizygous for elastin.
@nl
prefLabel
Novel arterial pathology in mice and humans hemizygous for elastin.
@en
Novel arterial pathology in mice and humans hemizygous for elastin.
@nl
P2093
P2860
P356
P1476
Novel arterial pathology in mice and humans hemizygous for elastin.
@en
P2093
Keating MT
P2860
P304
P356
10.1172/JCI4487
P407
P577
1998-11-01T00:00:00Z