Deficiency in microfibril-associated glycoprotein-1 leads to complex phenotypes in multiple organ systems
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Human eye development is characterized by coordinated expression of fibrillin isoformsGenetic and functional linkage between ADAMTS superfamily proteins and fibrillin-1: a novel mechanism influencing microfibril assembly and functionElastin in the LiverDisruption of murine Adamtsl4 results in zonular fiber detachment from the lens and in retinal pigment epithelium dedifferentiationMicrofibril-associated glycoprotein 2 (MAGP2) loss of function has pleiotropic effects in vivoAdamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia--a novel mouse model providing insights into geleophysic dysplasiaDefining elastic fiber interactions by molecular fishing: an affinity purification and mass spectrometry approach.Biogenesis and function of fibrillin assemblies.The extracellular matrix protein MAGP1 supports thermogenesis and protects against obesity and diabetes through regulation of TGF-βMicrofibril structure masks fibrillin-2 in postnatal tissuesMicrofibril-associated glycoprotein-1, an extracellular matrix regulator of bone remodeling.Immobilized metal affinity chromatography co-purifies TGF-β1 with histidine-tagged recombinant extracellular proteinsGenetic dissection of quantitative trait Loci for hemostasis and thrombosis on mouse chromosomes 11 and 5 using congenic and subcongenic strains.EMILIN2 regulates platelet activation, thrombus formation, and clot retraction.The matrix-binding domain of microfibril-associated glycoprotein-1 targets active connective tissue growth factor to a fibroblast-produced extracellular matrixOophorectomy-induced bone loss is attenuated in MAGP1-deficient miceLTBP-2 Has a Single High-Affinity Binding Site for FGF-2 and Blocks FGF-2-Induced Cell Proliferation.Biological Significance of Local TGF-β Activation in Liver Diseases.MAGP1, the extracellular matrix, and metabolism.Development, composition, and structural arrangements of the ciliary zonule of the mouseMarrow Adipose Tissue Expansion Coincides with Insulin Resistance in MAGP1-Deficient Mice.Molecular mechanisms of thoracic aortic dissection.Ocular phenotype of Fbn2-null mice.Extracellular microfibrils in vertebrate development and disease processes.Vascular extracellular matrix and arterial mechanics.The microfibril-associated glycoproteins (MAGPs) and the microfibrillar niche.Identification of Pax3 and Zic1 targets in the developing neural crest.Unusual life cycle and impact on microfibril assembly of ADAMTS17, a secreted metalloprotease mutated in genetic eye disease.Microfibril-associated glycoprotein-1 controls human ciliary zonule development in vitro.TB domain proteins: evolutionary insights into the multifaceted roles of fibrillins and LTBPs.Overview of extracellular matrix.Assembly of fibrillin microfibrils governs extracellular deposition of latent TGF beta.Characterization of metabolic health in mouse models of fibrillin-1 perturbation.Latent transforming growth factor beta-binding proteins and fibulins compete for fibrillin-1 and exhibit exquisite specificities in binding sites.Elastic Fibers and Large Artery Mechanics.Modulation of transforming growth factor-β/follistatin signaling and white adipose browning: therapeutic implications for obesity related disorders.Structural and compositional diversity of fibrillin microfibrils in human tissues.Simple and rapid characterization of novel large germline deletions in SDHB, SDHC and SDHD-related paraganglioma.Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease.Contribution of metabolic disease to bone fragility in MAGP1-deficient mice.
P2860
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P2860
Deficiency in microfibril-associated glycoprotein-1 leads to complex phenotypes in multiple organ systems
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
Deficiency in microfibril-asso ...... ypes in multiple organ systems
@en
type
label
Deficiency in microfibril-asso ...... ypes in multiple organ systems
@en
prefLabel
Deficiency in microfibril-asso ...... ypes in multiple organ systems
@en
P2093
P2860
P356
P1476
Deficiency in microfibril-asso ...... ypes in multiple organ systems
@en
P2093
Clarissa S Craft
Fernando Segade
Richard A Pierce
Robert P Mecham
Russell H Knutsen
Thomas J Broekelmann
P2860
P304
25533-25543
P356
10.1074/JBC.M709962200
P407
P577
2008-07-14T00:00:00Z