Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice.
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Differential regulation of elastic fiber formation by fibulin-4 and -5Latent TGF-beta-binding protein 2 binds to DANCE/fibulin-5 and regulates elastic fiber assemblyA novel intracellular fibulin-1D variant binds to the cytoplasmic domain of integrin beta 1 subunitFibulin-4: a novel gene for an autosomal recessive cutis laxa syndromeFibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblastsMatrix control of transforming growth factor-β functionElastin in the LiverCutis laxa: intersection of elastic fiber biogenesis, TGFβ signaling, the secretory pathway and metabolismCharacterization of a distinct lethal arteriopathy syndrome in twenty-two infants associated with an identical, novel mutation in FBLN4 gene, confirms fibulin-4 as a critical determinant of human vascular elastogenesisModeling autosomal recessive cutis laxa type 1C in mice reveals distinct functions for Ltbp-4 isoformsGenome-wide association analysis identifies six new loci associated with forced vital capacityThe extracellular matrix in development and morphogenesis: a dynamic view.Knockout mice: is it just genetics? Effect of enriched housing on fibulin-4(+/-) mice.Fibulin-2 and fibulin-5 cooperatively function to form the internal elastic lamina and protect from vascular injury.Early AMD-like defects in the RPE and retinal degeneration in aged mice with RPE-specific deletion of Atg5 or Atg7Fibulin-4 is essential for maintaining arterial wall integrity in conduit but not muscular arteries.Fibulin-4 deficiency results in ascending aortic aneurysms: a potential link between abnormal smooth muscle cell phenotype and aneurysm progressionFibroblast growth factor receptors control epithelial-mesenchymal interactions necessary for alveolar elastogenesis.Unraveling the mechanism of elastic fiber assembly: The roles of short fibulinsFibulin-4 deposition requires EMILIN-1 in the extracellular matrix of osteoblasts.Impaired vascular contractility and aortic wall degeneration in fibulin-4 deficient mice: effect of angiotensin II type 1 (AT1) receptor blockade.Measuring, reversing, and modeling the mechanical changes due to the absence of Fibulin-4 in mouse arteries.Fibulin-4 and fibulin-5 in elastogenesis and beyond: Insights from mouse and human studies.Extracellular matrix defects in aneurysmal Fibulin-4 mice predispose to lung emphysema.Elastogenic protein expression of a highly elastic murine spinal ligament: the ligamentum flavumVascular wall extracellular matrix proteins and vascular diseasesAltered TGFbeta signaling and cardiovascular manifestations in patients with autosomal recessive cutis laxa type I caused by fibulin-4 deficiency.Fibulin-4 and -5, but not Fibulin-2, are Associated with Tropoelastin Deposition in Elastin-Producing Cell Culture.Arterial tortuosity in genetic arteriopathies.Fibrillin-1 interactions with fibulins depend on the first hybrid domain and provide an adaptor function to tropoelastin.Control of lung development by latent TGF-β binding proteins.Localization of microfibrillar-associated protein 4 (MFAP4) in human tissues: clinical evaluation of serum MFAP4 and its association with various cardiovascular conditions.Compromised mutant EFEMP1 secretion associated with macular dystrophy remedied by proteostasis network alteration.Impaired mesenchymal cell function in Gata4 mutant mice leads to diaphragmatic hernias and primary lung defects.Pelvic organ prolapse in fibulin-5 knockout mice: pregnancy-induced changes in elastic fiber homeostasis in mouse vagina.Differences in genetic signaling, and not mechanical properties of the wall, are linked to ascending aortic aneurysms in fibulin-4 knockout miceAlternative splicing and tissue-specific elastin misassembly act as biological modifiers of human elastin gene frameshift mutations associated with dominant cutis laxa.Fibulin-4 E57K Knock-in Mice Recapitulate Cutaneous, Vascular and Skeletal Defects of Recessive Cutis Laxa 1B with both Elastic Fiber and Collagen Fibril Abnormalities.Loss of fibulin-4 disrupts collagen synthesis and maturation: implications for pathology resulting from EFEMP2 mutationsExtracellular matrix and the mechanics of large artery development.
P2860
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P2860
Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice.
description
2006 nî lūn-bûn
@nan
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
2006年论文
@zh
2006年论文
@zh-cn
name
Targeted disruption of fibulin ...... s perinatal lethality in mice.
@en
type
label
Targeted disruption of fibulin ...... s perinatal lethality in mice.
@en
prefLabel
Targeted disruption of fibulin ...... s perinatal lethality in mice.
@en
P2093
P2860
P1476
Targeted disruption of fibulin ...... s perinatal lethality in mice.
@en
P2093
Alan D Marmorstein
Barry C Starcher
Brian McKay
J Brett Stanton
Lihua Y Marmorstein
Masahito Horiguchi
Precious J McLaughlin
Qiuyun Chen
Robert Mecham
Thomas J Broekelmann
P2860
P304
P356
10.1128/MCB.26.5.1700-1709.2006
P407
P577
2006-03-01T00:00:00Z