Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice. - Wikidata - awgldk - TriplyDB

Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice.

Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice.

http://www.wikidata.org/entity/Q41890204

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Differential regulation of elastic fiber formation by fibulin-4 and -5 Latent TGF-beta-binding protein 2 binds to DANCE/fibulin-5 and regulates elastic fiber assembly A novel intracellular fibulin-1D variant binds to the cytoplasmic domain of integrin beta 1 subunit Fibulin-4: a novel gene for an autosomal recessive cutis laxa syndrome Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblasts Matrix control of transforming growth factor-β function Elastin in the Liver Cutis laxa: intersection of elastic fiber biogenesis, TGFβ signaling, the secretory pathway and metabolism Characterization 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 elastogenesis Modeling autosomal recessive cutis laxa type 1C in mice reveals distinct functions for Ltbp-4 isoforms Genome-wide association analysis identifies six new loci associated with forced vital capacity The 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 Atg7 Fibulin-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 progression Fibroblast growth factor receptors control epithelial-mesenchymal interactions necessary for alveolar elastogenesis.Unraveling the mechanism of elastic fiber assembly: The roles of short fibulins Fibulin-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 flavum Vascular wall extracellular matrix proteins and vascular diseases Altered 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 mice Alternative 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 mutations Extracellular matrix and the mechanics of large artery development.

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P2860

Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice.

http://www.wikidata.org/entity/Q41890204

description

2006 nî lūn-bûn

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Targeted disruption of fibulin ...... s perinatal lethality in mice.

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Targeted disruption of fibulin ...... s perinatal lethality in mice.

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Targeted disruption of fibulin ...... s perinatal lethality in mice.

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MCB.26.5.1700-1709.2006

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Molecular and Cellular Biology

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Targeted disruption of fibulin ...... s perinatal lethality in mice.

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Alan D Marmorstein

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Barry C Starcher

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Brian McKay

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J Brett Stanton

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Lihua Y Marmorstein

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Masahito Horiguchi

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Precious J McLaughlin

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Qiuyun Chen

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Robert Mecham

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Thomas J Broekelmann

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P2860

A single EFEMP1 mutation associated with both Malattia Leventinese and Doyne honeycomb retinal dystrophy

Sequence, recombinant expression and tissue localization of two novel extracellular matrix proteins, fibulin-3 and fibulin-4

Bestrophin, the product of the Best vitelliform macular dystrophy gene (VMD2), localizes to the basolateral plasma membrane of the retinal pigment epithelium

Cloning and characterization of a fourth human lysyl oxidase isoenzyme

Fibulin-5 is an elastin-binding protein essential for elastic fibre development in vivo

Elastin is an essential determinant of arterial morphogenesis

Genetic heterogeneity of cutis laxa: a heterozygous tandem duplication within the fibulin-5 (FBLN5) gene.

Aberrant accumulation of EFEMP1 underlies drusen formation in Malattia Leventinese and age-related macular degeneration

Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1

Regulation of limb patterning by extracellular microfibrils

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