Published in Curr Opin Cell Biol on June 12, 2009
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Deciphering the genetic architecture of variation in the immune response to Mycobacterium tuberculosis infection. Proc Natl Acad Sci U S A (2012) 1.73
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A novel missense mutation in ADAMTS10 in Norwegian Elkhound primary glaucoma. PLoS One (2014) 1.48
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The microfibril hypothesis of glaucoma: implications for treatment of elevated intraocular pressure. J Ocul Pharmacol Ther (2014) 1.03
Integration of planar cell polarity and ECM signaling in elongation of the vertebrate body plan. Curr Opin Cell Biol (2010) 1.03
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Targeting TGFβ signaling in subchondral bone and articular cartilage homeostasis. Trends Pharmacol Sci (2014) 0.96
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Dexamethasone Stiffens Trabecular Meshwork, Trabecular Meshwork Cells, and Matrix. Invest Ophthalmol Vis Sci (2015) 0.86
Insights into the osteoblast precursor differentiation towards mature osteoblasts induced by continuous BMP-2 signaling. Biol Open (2013) 0.85
ADAMTSL6β protein rescues fibrillin-1 microfibril disorder in a Marfan syndrome mouse model through the promotion of fibrillin-1 assembly. J Biol Chem (2011) 0.85
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Putative functions of extracellular matrix glycoproteins in secondary palate morphogenesis. Front Physiol (2012) 0.83
Screening ADAMTS10 in dog populations supports Gly661Arg as the glaucoma-causing variant in beagles. Invest Ophthalmol Vis Sci (2013) 0.83
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Drosophila MMP2 regulates the matrix molecule faulty attraction (Frac) to promote motor axon targeting in Drosophila. J Neurosci (2011) 0.82
SMAD4 mutations causing Myhre syndrome result in disorganization of extracellular matrix improved by losartan. Eur J Hum Genet (2014) 0.81
Immunolocalisation of fibrillin microfibrils in the calf metacarpal and vertebral growth plate. J Anat (2013) 0.81
Generation of Fbn1 conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment. Genesis (2012) 0.81
Fibrillin microfibrils in bone physiology. Matrix Biol (2015) 0.79
Molecular Mechanism Responsible for Fibronectin-controlled Alterations in Matrix Stiffness in Advanced Chronic Liver Fibrogenesis. J Biol Chem (2015) 0.79
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. J Biol Chem (2015) 0.79
Insight into pathologic abnormalities in congenital semilunar valve disease based on advances in understanding normal valve microstructure and extracellular matrix. Cardiovasc Pathol (2011) 0.79
Rapid Activation of Bone Morphogenic Protein 9 by Receptor-mediated Displacement of Pro-domains. J Biol Chem (2015) 0.78
Establishment of fibrillin-deficient osteoprogenitor cell lines identifies molecular abnormalities associated with extracellular matrix perturbation of osteogenic differentiation. Cell Tissue Res (2011) 0.78
Mechanical Forces Reshape Differentiation Cues That Guide Cardiomyogenesis. Circ Res (2016) 0.78
Corneal stroma microfibrils. Exp Eye Res (2015) 0.78
Simultaneous rather than ordered cleavage of two sites within the BMP4 prodomain leads to loss of ligand in mice. Development (2014) 0.78
Drug-based therapies for vascular disease in Marfan syndrome: from mouse models to human patients. Mt Sinai J Med (2010) 0.78
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Extracellular Regulation of Bone Morphogenetic Protein Activity by the Microfibril Component Fibrillin-1. J Biol Chem (2016) 0.77
Elevated transforming growth factor β1 in plasma of primary open-angle glaucoma patients. Invest Ophthalmol Vis Sci (2014) 0.77
NMR spectroscopic and bioinformatic analyses of the LTBP1 C-terminus reveal a highly dynamic domain organisation. PLoS One (2014) 0.77
The prodomain of BMP4 is necessary and sufficient to generate stable BMP4/7 heterodimers with enhanced bioactivity in vivo. Proc Natl Acad Sci U S A (2015) 0.76
Diagnostic Exome Sequencing Identifies a Novel Gene, EMILIN1, Associated with Autosomal-Dominant Hereditary Connective Tissue Disease. Hum Mutat (2015) 0.76
Syndecan4 coordinates Wnt/JNK and BMP signaling to regulate foregut progenitor development. Dev Biol (2016) 0.76
LTBP-2 Has a Single High-Affinity Binding Site for FGF-2 and Blocks FGF-2-Induced Cell Proliferation. PLoS One (2015) 0.76
Fibrillins in Tendon. Front Aging Neurosci (2016) 0.75
Fibrillin-1 microfibrils influence adult bone marrow hematopoiesis. Matrix Biol (2015) 0.75
Multigenic Delineation of Lower Jaw Deformity in Triploid Atlantic Salmon (Salmo salar L.). PLoS One (2016) 0.75
ADAMTS-10 and -6 differentially regulate cell-cell junctions and focal adhesions. Sci Rep (2016) 0.75
Bone matrix to growth factors: location, location, location. J Cell Biol (2010) 0.75
Endoplasmic Reticulum Oxidative Stress Triggers Tgf-Beta-Dependent Muscle Dysfunction by Accelerating Ascorbic Acid Turnover. Sci Rep (2017) 0.75
DPY-17 and MUA-3 Interact for Connective Tissue-Like Tissue Integrity in Caenorhabditis elegans: A Model for Marfan Syndrome. G3 (Bethesda) (2015) 0.75
Supramolecular Nanofibers Enhance Growth Factor Signaling by Increasing Lipid Raft Mobility. Nano Lett (2016) 0.75
The integrin alpha v beta 6 binds and activates latent TGF beta 1: a mechanism for regulating pulmonary inflammation and fibrosis. Cell (1999) 11.59
Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome. Nat Genet (2003) 11.01
Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science (2006) 10.25
Angiotensin II blockade and aortic-root dilation in Marfan's syndrome. N Engl J Med (2008) 7.31
Myofibroblast contraction activates latent TGF-beta1 from the extracellular matrix. J Cell Biol (2007) 5.60
Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states. Nat Med (2007) 5.25
Mutations in smooth muscle alpha-actin (ACTA2) lead to thoracic aortic aneurysms and dissections. Nat Genet (2007) 4.93
TGF-beta-dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome. J Clin Invest (2004) 4.85
The integrin alpha(v)beta8 mediates epithelial homeostasis through MT1-MMP-dependent activation of TGF-beta1. J Cell Biol (2002) 4.60
Heparan sulphate proteoglycans: the sweet side of development. Nat Rev Mol Cell Biol (2005) 3.92
Mutations in myosin heavy chain 11 cause a syndrome associating thoracic aortic aneurysm/aortic dissection and patent ductus arteriosus. Nat Genet (2006) 3.65
Latent transforming growth factor-beta (TGF-beta) binding proteins: orchestrators of TGF-beta availability. J Biol Chem (2004) 2.97
Absence of integrin-mediated TGFbeta1 activation in vivo recapitulates the phenotype of TGFbeta1-null mice. J Cell Biol (2007) 2.94
Integrin alphaVbeta6-mediated activation of latent TGF-beta requires the latent TGF-beta binding protein-1. J Cell Biol (2004) 2.90
Integrins and the activation of latent transforming growth factor beta1 - an intimate relationship. Eur J Cell Biol (2008) 2.74
Fibronectin polymerization regulates the composition and stability of extracellular matrix fibrils and cell-matrix adhesions. Mol Biol Cell (2002) 2.62
Type IV collagens regulate BMP signalling in Drosophila. Nature (2008) 2.50
Regulation of limb patterning by extracellular microfibrils. J Cell Biol (2001) 2.37
Perturbations of vascular homeostasis and aortic valve abnormalities in fibulin-4 deficient mice. Circ Res (2007) 2.32
Fibrillins 1 and 2 perform partially overlapping functions during aortic development. J Biol Chem (2005) 2.19
BMP1 controls TGFbeta1 activation via cleavage of latent TGFbeta-binding protein. J Cell Biol (2006) 2.17
Disruption of the gene encoding the latent transforming growth factor-beta binding protein 4 (LTBP-4) causes abnormal lung development, cardiomyopathy, and colorectal cancer. Genes Dev (2002) 2.13
Fibronectin regulates latent transforming growth factor-beta (TGF beta) by controlling matrix assembly of latent TGF beta-binding protein-1. J Biol Chem (2005) 1.82
Targeting of bone morphogenetic protein growth factor complexes to fibrillin. J Biol Chem (2008) 1.80
Long-term doxycycline is more effective than atenolol to prevent thoracic aortic aneurysm in marfan syndrome through the inhibition of matrix metalloproteinase-2 and -9. Circ Res (2008) 1.67
The role of proteases in transforming growth factor-beta activation. Int J Biochem Cell Biol (2007) 1.67
Fibrillin-1 regulates the bioavailability of TGFbeta1. J Cell Biol (2007) 1.67
Fibrillin assembly requires fibronectin. Mol Biol Cell (2008) 1.53
Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome. J Vasc Surg (2008) 1.48
LRP1 functions as an atheroprotective integrator of TGFbeta and PDFG signals in the vascular wall: implications for Marfan syndrome. PLoS One (2007) 1.44
Interactions of fibrillin-1 with heparin/heparan sulfate, implications for microfibrillar assembly. J Biol Chem (2001) 1.42
Perturbation of transforming growth factor (TGF)-beta1 association with latent TGF-beta binding protein yields inflammation and tumors. Proc Natl Acad Sci U S A (2008) 1.40
Premature termination mutations in FBN1: distinct effects on differential allelic expression and on protein and clinical phenotypes. Am J Hum Genet (2002) 1.38
p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice. J Biol Chem (2008) 1.32
Potential role for heparan sulfate proteoglycans in regulation of transforming growth factor-beta (TGF-beta) by modulating assembly of latent TGF-beta-binding protein-1. J Biol Chem (2007) 1.32
Fibrillin-1 microfibril deposition is dependent on fibronectin assembly. J Cell Sci (2008) 1.30
New insights into extracellular matrix assembly and reorganization from dynamic imaging of extracellular matrix proteins in living osteoblasts. J Cell Sci (2006) 1.28
Fibronectin and heparin binding domains of latent TGF-beta binding protein (LTBP)-4 mediate matrix targeting and cell adhesion. Exp Cell Res (2008) 1.21
Extracellular microfibrils in development and disease. Cell Mol Life Sci (2007) 1.21
A new model for growth factor activation: type II receptors compete with the prodomain for BMP-7. J Mol Biol (2008) 1.21
Induction of macrophage chemotaxis by aortic extracts of the mgR Marfan mouse model and a GxxPG-containing fibrillin-1 fragment. Circulation (2006) 1.20
Fibrillin-1 interactions with heparin. Implications for microfibril and elastic fiber assembly. J Biol Chem (2005) 1.19
Dual functions for LTBP in lung development: LTBP-4 independently modulates elastogenesis and TGF-beta activity. J Cell Physiol (2009) 1.14
Biogenesis of extracellular microfibrils: Multimerization of the fibrillin-1 C terminus into bead-like structures enables self-assembly. Proc Natl Acad Sci U S A (2008) 1.14
RGD-containing fibrillin-1 fragments upregulate matrix metalloproteinase expression in cell culture: a potential factor in the pathogenesis of the Marfan syndrome. Hum Genet (2004) 1.12
Cell adhesion to fibrillin-1: identification of an Arg-Gly-Asp-dependent synergy region and a heparin-binding site that regulates focal adhesion formation. J Cell Sci (2007) 1.08
Fibrillin-1 and -2 contain heparin-binding sites important for matrix deposition and that support cell attachment. Biochem J (2003) 1.07
Large genomic fibrillin-1 (FBN1) gene deletions provide evidence for true haploinsufficiency in Marfan syndrome. Hum Genet (2007) 1.07
Expression of truncated latent TGF-beta-binding protein modulates TGF-beta signaling. J Cell Sci (2005) 1.02
Heparan sulfate regulates fibrillin-1 N- and C-terminal interactions. J Biol Chem (2008) 1.01
Specific microbiota direct the differentiation of IL-17-producing T-helper cells in the mucosa of the small intestine. Cell Host Microbe (2008) 11.08
Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science (2006) 10.25
A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet (2005) 9.62
Making sense of latent TGFbeta activation. J Cell Sci (2003) 8.90
Myofibroblast contraction activates latent TGF-beta1 from the extracellular matrix. J Cell Biol (2007) 5.60
Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-associated protein. J Biol Chem (2002) 3.92
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In vitro and in vivo evidence for shear-induced activation of latent transforming growth factor-beta1. Blood (2008) 1.94
Latent transforming growth factor beta-binding proteins and fibulins compete for fibrillin-1 and exhibit exquisite specificities in binding sites. J Biol Chem (2009) 1.73
The integrin alphaVbeta6 binds and activates latent TGFbeta3. FEBS Lett (2002) 1.64
Fibronectin is required for integrin alphavbeta6-mediated activation of latent TGF-beta complexes containing LTBP-1. FASEB J (2005) 1.61
GPR15-mediated homing controls immune homeostasis in the large intestine mucosa. Science (2013) 1.58
Isoform-specific activation of latent transforming growth factor beta (LTGF-beta) by reactive oxygen species. Radiat Res (2006) 1.47
A rapid and sensitive bioassay to measure bone morphogenetic protein activity. BMC Cell Biol (2007) 1.45
Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology. Physiol Genomics (2004) 1.45
Perturbation of transforming growth factor (TGF)-beta1 association with latent TGF-beta binding protein yields inflammation and tumors. Proc Natl Acad Sci U S A (2008) 1.40
Matrix control of transforming growth factor-β function. J Biochem (2012) 1.39
LTBPs, more than just an escort service. J Cell Biochem (2012) 1.37
Bone abnormalities in latent TGF-[beta] binding protein (Ltbp)-3-null mice indicate a role for Ltbp-3 in modulating TGF-[beta] bioavailability. J Cell Biol (2002) 1.37
Mutations in LTBP4 cause a syndrome of impaired pulmonary, gastrointestinal, genitourinary, musculoskeletal, and dermal development. Am J Hum Genet (2009) 1.34
p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice. J Biol Chem (2008) 1.32
Cell signaling events: a view from the matrix. Matrix Biol (2003) 1.26
Long form of latent TGF-beta binding protein 1 (Ltbp1L) is essential for cardiac outflow tract septation and remodeling. Development (2007) 1.22
Specificity of latent TGF-β binding protein (LTBP) incorporation into matrix: role of fibrillins and fibronectin. J Cell Physiol (2012) 1.22
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Latent TGF-β binding protein 4 promotes elastic fiber assembly by interacting with fibulin-5. Proc Natl Acad Sci U S A (2013) 1.04
Microfibril structure masks fibrillin-2 in postnatal tissues. J Biol Chem (2010) 1.03
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Mechanisms of tendon injury and repair. J Orthop Res (2015) 1.02
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Solution structure of the third TB domain from LTBP1 provides insight into assembly of the large latent complex that sequesters latent TGF-beta. J Mol Biol (2003) 0.99
Amino acid requirements for formation of the TGF-beta-latent TGF-beta binding protein complexes. J Mol Biol (2005) 0.98
An osteoblast-derived proteinase controls tumor cell survival via TGF-beta activation in the bone microenvironment. PLoS One (2012) 0.95
Latent transforming growth factor-beta-binding protein-4 regulates transforming growth factor-beta1 bioavailability for activation by fibrogenic lung fibroblasts in response to bleomycin. Am J Pathol (2008) 0.95
Latent TGF-beta binding protein-3 (LTBP-3) requires binding to TGF-beta for secretion. FEBS Lett (2002) 0.95
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F-spondin, a neuroregulatory protein, is up-regulated in osteoarthritis and regulates cartilage metabolism via TGF-beta activation. FASEB J (2008) 0.93
Annexin II-mediated plasmin generation activates TGF-beta3 during epithelial-mesenchymal transformation in the developing avian heart. Dev Biol (2004) 0.90
Long form of latent TGF-β binding protein 1 (Ltbp1L) regulates cardiac valve development. Dev Dyn (2011) 0.88
Thrombospondin-1 (TSP-1) analogs ABT-510 and ABT-898 inhibit prolactinoma growth and recover active pituitary transforming growth factor-β1 (TGF-β1). Endocrinology (2012) 0.88
Active and total transforming growth factor-β1 are differentially regulated by dopamine and estradiol in the pituitary. Endocrinology (2011) 0.88
Function of latent TGFβ binding protein 4 and fibulin 5 in elastogenesis and lung development. J Cell Physiol (2015) 0.87
Control of lung development by latent TGF-β binding proteins. J Cell Physiol (2011) 0.87
Growth retardation as well as spleen and thymus involution in latent TGF-beta binding protein (Ltbp)-3 null mice. J Cell Physiol (2003) 0.86
The latent-TGFbeta-binding-protein-1 (LTBP-1) is expressed in the organizer and regulates nodal and activin signaling. Dev Biol (2002) 0.84
Production of gastrointestinal tumors in mice by modulating latent TGF-β1 activation. Cancer Res (2012) 0.83
Molecular cloning of the mouse Ltbp-1 gene reveals tissue specific expression of alternatively spliced forms. Gene (2003) 0.82
A genetic screen to identify latent transforming growth factor beta activators. Anal Biochem (2004) 0.81
Overexpression of the 18 kDa and 22/24 kDa FGF-2 isoforms results in differential drug resistance and amplification potential. J Cell Physiol (2002) 0.79
Sex differences in the pituitary transforming growth factor-β1 system: studies in a model of resistant prolactinomas. Endocrinology (2013) 0.78
Genetic suppression of inflammation blocks the tumor-promoting effects of TGF-β in gastric tissue. Cancer Res (2014) 0.77
Is losartan the drug for all seasons? Curr Opin Pharmacol (2012) 0.76