about
Genetic and functional linkage between ADAMTS superfamily proteins and fibrillin-1: a novel mechanism influencing microfibril assembly and functionMatrix control of transforming growth factor-β functionAbnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null MiceMicroenvironmental regulation by fibrillin-1Characterization of Microfibrillar-associated Protein 4 (MFAP4) as a Tropoelastin- and Fibrillin-binding Protein Involved in Elastic Fiber FormationGeneration of heterozygous fibrillin-1 mutant cloned pigs from genome-edited foetal fibroblastsExtracellular matrix molecules facilitating vascular biointegrationThe Pathogenic Transforming Growth Factor-β Overdrive Hypothesis in Aortic Aneurysms and Dissections: A Mirage?The organisation of elastin and fibrillins 1 and 2 in the cruciate ligament complex.Fibulin-4 deposition requires EMILIN-1 in the extracellular matrix of osteoblasts.C-terminal propeptide is required for fibrillin-1 secretion and blocks premature assembly through linkage to domains cbEGF41-43.Altered tissue behavior of a non-aneurysmal descending thoracic aorta in the mouse model of Marfan syndrome.Fibrillin assemblies: extracellular determinants of tissue formation and fibrosisControl of lung development by latent TGF-β binding proteins.Skeletogenic phenotype of human Marfan embryonic stem cells faithfully phenocopied by patient-specific induced-pluripotent stem cellsLTBP-2 Has a Single High-Affinity Binding Site for FGF-2 and Blocks FGF-2-Induced Cell Proliferation.Specificity of latent TGF-β binding protein (LTBP) incorporation into matrix: role of fibrillins and fibronectinFibrillin-containing microfibrils are key signal relay stations for cell function.Molecular mechanisms of thoracic aortic dissection.Homotypic versican G1 domain interactions enhance hyaluronan incorporation into fibrillin microfibrilsTB domain proteins: evolutionary insights into the multifaceted roles of fibrillins and LTBPs.TGF-β and the TGF-β Family: Context-Dependent Roles in Cell and Tissue Physiology.Genes in Thoracic Aortic Aneurysms and Dissections - Do they Matter?: Translation and Integration of Research and Modern Genetic Techniques into Daily Clinical Practice.Sex, pregnancy and aortic disease in Marfan syndrome.The N-Terminal Region of Fibrillin-1 Mediates a Bipartite Interaction with LTBP1.Characterization of metabolic health in mouse models of fibrillin-1 perturbation.Fell-Muir Lecture: Fibrillin microfibrils: structural tensometers of elastic tissues?Early fibrillin-1 assembly monitored through a modifiable recombinant cell approach.FGF receptors control alveolar elastogenesis.Intrinsic cardiomyopathy in Marfan syndrome: results from in-vivo and ex-vivo studies of the Fbn1C1039G/+ model and longitudinal findings in humans.Marfan and Sartans: time to wake up!
P2860
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P2860
description
2010 nî lūn-bûn
@nan
2010 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
In vivo studies of mutant fibrillin-1 microfibrils
@ast
In vivo studies of mutant fibrillin-1 microfibrils
@en
type
label
In vivo studies of mutant fibrillin-1 microfibrils
@ast
In vivo studies of mutant fibrillin-1 microfibrils
@en
prefLabel
In vivo studies of mutant fibrillin-1 microfibrils
@ast
In vivo studies of mutant fibrillin-1 microfibrils
@en
P2093
P2860
P356
P1476
In vivo studies of mutant fibrillin-1 microfibrils
@en
P2093
Douglas R Keene
Elise C Manalo
Eric J Carlson
Francesco Ramirez
Gerhard Sengle
Lynn Y Sakai
Noe L Charbonneau
Valerie M Carlberg
P2860
P304
24943-24955
P356
10.1074/JBC.M110.130021
P407
P577
2010-06-07T00:00:00Z