Matrix-dependent perturbation of TGFβ signaling and disease.

PubWeight™: 1.31‹?› | Rank: Top 10%

🔗 View Article (PMC 3426037)

Published in FEBS Lett on May 26, 2012

Authors

Jefferson J Doyle1, Elizabeth E Gerber, Harry C Dietz

Author Affiliations

1: Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA. jefferson.doyle@jhmi.edu

Articles citing this

Integrin-modulating therapy prevents fibrosis and autoimmunity in mouse models of scleroderma. Nature (2013) 1.86

Prestress in the extracellular matrix sensitizes latent TGF-β1 for activation. J Cell Biol (2014) 1.70

Myofibroblasts: trust your heart and let fate decide. J Mol Cell Cardiol (2013) 1.25

Cerebral small vessel disease-related protease HtrA1 processes latent TGF-β binding protein 1 and facilitates TGF-β signaling. Proc Natl Acad Sci U S A (2014) 1.19

Unchaining the beast; insights from structural and evolutionary studies on TGFβ secretion, sequestration, and activation. Cytokine Growth Factor Rev (2013) 1.08

The biology of the extracellular matrix: novel insights. Curr Opin Rheumatol (2013) 1.06

Role of mechanotransduction in vascular biology: focus on thoracic aortic aneurysms and dissections. Circ Res (2015) 1.04

Intricate interplay between astrocytes and motor neurons in ALS. Proc Natl Acad Sci U S A (2013) 0.97

Mechanotransduction and fibrosis. J Biomech (2014) 0.97

Modifying muscular dystrophy through transforming growth factor-β. FEBS J (2013) 0.95

Tumor suppressor ataxia telangiectasia mutated functions downstream of TGF-β1 in orchestrating profibrotic responses. FASEB J (2014) 0.94

The significance of macrophage polarization subtypes for animal models of tissue fibrosis and human fibrotic diseases. Clin Transl Med (2015) 0.90

The pathology and pathobiology of bicuspid aortic valve: State of the art and novel research perspectives. J Pathol Clin Res (2015) 0.87

Sequestration of latent TGF-β binding protein 1 into CADASIL-related Notch3-ECD deposits. Acta Neuropathol Commun (2014) 0.85

Pharmacologically Improved Contractility Protects Against Aortic Dissection in Mice With Disrupted Transforming Growth Factor-β Signaling Despite Compromised Extracellular Matrix Properties. Arterioscler Thromb Vasc Biol (2016) 0.82

MicroRNA miR145 regulates TGFBR2 expression and matrix synthesis in vascular smooth muscle cells. Circ Res (2014) 0.82

Transforming growth factor-beta (TGF- β) signaling in paravertebral muscles in juvenile and adolescent idiopathic scoliosis. Biomed Res Int (2014) 0.82

Advancing biomaterials of human origin for tissue engineering. Prog Polym Sci (2015) 0.81

SMAD4 mutations causing Myhre syndrome result in disorganization of extracellular matrix improved by losartan. Eur J Hum Genet (2014) 0.81

Identification of candidate downstream targets of TGFβ signaling during palate development by genome-wide transcript profiling. J Cell Biochem (2013) 0.81

Interleukin-1-induced changes in the glioblastoma secretome suggest its role in tumor progression. J Proteomics (2014) 0.81

Medical treatment of aortic aneurysms in Marfan syndrome and other heritable conditions. Curr Cardiol Rev (2014) 0.81

Fibrillin-1 directly regulates osteoclast formation and function by a dual mechanism. J Cell Sci (2013) 0.81

Smooth muscle cell-specific Tgfbr1 deficiency promotes aortic aneurysm formation by stimulating multiple signaling events. Sci Rep (2016) 0.80

Cardiovascular Benefits of Moderate Exercise Training in Marfan Syndrome: Insights From an Animal Model. J Am Heart Assoc (2017) 0.80

Small leucine-rich proteoglycans exhibit unique spatiotemporal expression profiles during cardiac valve development. Dev Dyn (2014) 0.79

Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia--a novel mouse model providing insights into geleophysic dysplasia. Dis Model Mech (2015) 0.79

A potential role for endogenous proteins as sacrificial sunscreens and antioxidants in human tissues. Redox Biol (2015) 0.78

Genetic analysis of the contribution of LTBP-3 to thoracic aneurysm in Marfan syndrome. Proc Natl Acad Sci U S A (2015) 0.77

LTBP-2 Has a Single High-Affinity Binding Site for FGF-2 and Blocks FGF-2-Induced Cell Proliferation. PLoS One (2015) 0.76

The pituitary TGFβ1 system as a novel target for the treatment of resistant prolactinomas. J Endocrinol (2015) 0.76

An iPSC-derived vascular model of Marfan syndrome identifies key mediators of smooth muscle cell death. Nat Genet (2016) 0.75

Association between Fibrillin1 Polymorphisms (rs2118181, rs10519177) and Transforming Growth Factor β1 Concentration in Human Plasma. Mol Med (2015) 0.75

Correlation between Fibrillin-1 Degradation and mRNA Downregulation and Myofibroblast Differentiation in Cultured Human Dental Pulp Tissue. J Histochem Cytochem (2015) 0.75

Aortic aneurysm. J Transl Int Med (2016) 0.75

The N-Terminal Region of Fibrillin-1 Mediates a Bipartite Interaction with LTBP1. Structure (2017) 0.75

Pathogenic Mechanisms of Bicuspid Aortic Valve Aortopathy. Front Physiol (2017) 0.75

Articles cited by this

(truncated to the top 100)

Smad-dependent and Smad-independent pathways in TGF-beta family signalling. Nature (2003) 20.48

Myofibroblasts and mechano-regulation of connective tissue remodelling. Nat Rev Mol Cell Biol (2002) 14.80

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

A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet (2005) 9.62

Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med (2006) 9.50

Angiotensin II blockade and aortic-root dilation in Marfan's syndrome. N Engl J Med (2008) 7.31

Heterozygous TGFBR2 mutations in Marfan syndrome. Nat Genet (2004) 6.15

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 regulation of TGFbeta signal transduction. Development (2009) 4.59

Activin receptor-like kinase 1 modulates transforming growth factor-beta 1 signaling in the regulation of angiogenesis. Proc Natl Acad Sci U S A (2000) 4.08

Mutations in SMAD3 cause a syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis. Nat Genet (2011) 3.93

Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-associated protein. J Biol Chem (2002) 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

Regression of abdominal aortic aneurysm by inhibition of c-Jun N-terminal kinase. Nat Med (2005) 3.59

Noncanonical TGFβ signaling contributes to aortic aneurysm progression in Marfan syndrome mice. Science (2011) 3.49

Transforming growth factor-beta function blocking prevents myocardial fibrosis and diastolic dysfunction in pressure-overloaded rats. Circulation (2002) 3.20

Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism. Science (2011) 3.18

TRAF6 mediates Smad-independent activation of JNK and p38 by TGF-beta. Mol Cell (2008) 3.04

Lessons on the pathogenesis of aneurysm from heritable conditions. Nature (2011) 2.97

Mutations in the facilitative glucose transporter GLUT10 alter angiogenesis and cause arterial tortuosity syndrome. Nat Genet (2006) 2.94

An adventitial IL-6/MCP1 amplification loop accelerates macrophage-mediated vascular inflammation leading to aortic dissection in mice. J Clin Invest (2009) 2.86

Assembly of fibronectin extracellular matrix. Annu Rev Cell Dev Biol (2010) 2.86

Emilin1 links TGF-beta maturation to blood pressure homeostasis. Cell (2006) 2.84

Mutations in transforming growth factor-beta receptor type II cause familial thoracic aortic aneurysms and dissections. Circulation (2005) 2.77

Integrins and the activation of latent transforming growth factor beta1 - an intimate relationship. Eur J Cell Biol (2008) 2.74

Circulating transforming growth factor-beta in Marfan syndrome. Circulation (2009) 2.69

Mutations in myosin light chain kinase cause familial aortic dissections. Am J Hum Genet (2010) 2.67

Neurofibromin regulation of ERK signaling modulates GABA release and learning. Cell (2008) 2.65

Signal co-operation between integrins and other receptor systems. Biochem J (2009) 2.59

TGF-beta activates Erk MAP kinase signalling through direct phosphorylation of ShcA. EMBO J (2007) 2.57

Angiotensin II activates the Smad pathway in vascular smooth muscle cells by a transforming growth factor-beta-independent mechanism. Circulation (2005) 2.55

Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome. Sci Transl Med (2010) 2.55

The myofibroblast: paradigm for a mechanically active cell. J Biomech (2009) 2.53

Endoglin, an ancillary TGFbeta receptor, is required for extraembryonic angiogenesis and plays a key role in heart development. Dev Biol (2000) 2.50

A disintegrin-like and metalloprotease (reprolysin-type) with thrombospondin type 1 motif (ADAMTS) superfamily: functions and mechanisms. J Biol Chem (2009) 2.49

Null mutations in LTBP2 cause primary congenital glaucoma. Am J Hum Genet (2009) 2.35

Transforming growth factor (TGF)-β signaling in cardiac remodeling. J Mol Cell Cardiol (2010) 2.35

Integrin-TGF-beta crosstalk in fibrosis, cancer and wound healing. EMBO Rep (2010) 2.32

Perturbations of vascular homeostasis and aortic valve abnormalities in fibulin-4 deficient mice. Circ Res (2007) 2.32

TGF-beta activity protects against inflammatory aortic aneurysm progression and complications in angiotensin II-infused mice. J Clin Invest (2010) 2.32

In frame fibrillin-1 gene deletion in autosomal dominant Weill-Marchesani syndrome. J Med Genet (2003) 2.26

ADAMTS10 mutations in autosomal recessive Weill-Marchesani syndrome. Am J Hum Genet (2004) 2.22

MYH11 mutations result in a distinct vascular pathology driven by insulin-like growth factor 1 and angiotensin II. Hum Mol Genet (2007) 2.17

Rationale and design of a randomized clinical trial of beta-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome. Am Heart J (2007) 2.14

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

Exome sequencing identifies SMAD3 mutations as a cause of familial thoracic aortic aneurysm and dissection with intracranial and other arterial aneurysms. Circ Res (2011) 1.93

An essential role for fibronectin extra type III domain A in pulmonary fibrosis. Am J Respir Crit Care Med (2007) 1.91

VEGF-integrin interplay controls tumor growth and vascularization. Proc Natl Acad Sci U S A (2005) 1.84

Angiotensin receptor regulates cardiac hypertrophy and transforming growth factor-beta 1 expression. Hypertension (1994) 1.83

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

Shared expression of phenotypic markers in systemic sclerosis indicates a convergence of pericytes and fibroblasts to a myofibroblast lineage in fibrosis. Arthritis Res Ther (2005) 1.81

Increased expression of integrin alpha(v)beta3 contributes to the establishment of autocrine TGF-beta signaling in scleroderma fibroblasts. J Immunol (2005) 1.81

Usefulness of enalapril versus propranolol or atenolol for prevention of aortic dilation in patients with the Marfan syndrome. Am J Cardiol (2005) 1.81

Alterations of beta-adrenergic signaling and cardiac hypertrophy in transgenic mice overexpressing TGF-beta(1). Am J Physiol Heart Circ Physiol (2002) 1.79

Transforming growth factor beta as a therapeutic target in systemic sclerosis. Nat Rev Rheumatol (2009) 1.79

Angiotensin II induces thrombospondin-1 production in human mesangial cells via p38 MAPK and JNK: a mechanism for activation of latent TGF-beta1. Am J Physiol Renal Physiol (2003) 1.79

ADAMTSL2 mutations in geleophysic dysplasia demonstrate a role for ADAMTS-like proteins in TGF-beta bioavailability regulation. Nat Genet (2008) 1.78

Homozygous mutations in ADAMTS10 and ADAMTS17 cause lenticular myopia, ectopia lentis, glaucoma, spherophakia, and short stature. Am J Hum Genet (2009) 1.73

Retracted Effect of perindopril on large artery stiffness and aortic root diameter in patients with Marfan syndrome: a randomized controlled trial. JAMA (2007) 1.69

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

TGF-beta in the pathogenesis and prevention of disease: a matter of aneurysmic proportions. J Clin Invest (2010) 1.65

Angiotensin II type 2 receptor mediates vascular smooth muscle cell apoptosis in cystic medial degeneration associated with Marfan's syndrome. Circulation (2001) 1.63

A homozygous mutation in ADAMTSL4 causes autosomal-recessive isolated ectopia lentis. Am J Hum Genet (2009) 1.63

Context-specific effects of fibulin-5 (DANCE/EVEC) on cell proliferation, motility, and invasion. Fibulin-5 is induced by transforming growth factor-beta and affects protein kinase cascades. J Biol Chem (2002) 1.58

Filamin A mutations cause periventricular heterotopia with Ehlers-Danlos syndrome. Neurology (2005) 1.57

Fibrillin-2 (FBN2) mutations result in the Marfan-like disorder, congenital contractural arachnodactyly. Nat Genet (1995) 1.57

Mutations in the TGFβ binding-protein-like domain 5 of FBN1 are responsible for acromicric and geleophysic dysplasias. Am J Hum Genet (2011) 1.55

Angiotensin II stimulates expression of transforming growth factor beta receptor type II in cultured mouse proximal tubular cells. J Mol Med (Berl) (1999) 1.54

Angiotensin II upregulates transforming growth factor-beta type I receptor on rat vascular smooth muscle cells. Am J Hypertens (2000) 1.54

Fibrillin assembly requires fibronectin. Mol Biol Cell (2008) 1.53

De novo ACTA2 mutation causes a novel syndrome of multisystemic smooth muscle dysfunction. Am J Med Genet A (2010) 1.49

Filamin associates with Smads and regulates transforming growth factor-beta signaling. J Biol Chem (2001) 1.48

Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome. J Vasc Surg (2008) 1.48

Lysyl oxidase binds transforming growth factor-beta and regulates its signaling via amine oxidase activity. J Biol Chem (2008) 1.47

Competition for talin results in trans-dominant inhibition of integrin activation. J Biol Chem (2004) 1.46

Fibulin-4 deficiency results in ascending aortic aneurysms: a potential link between abnormal smooth muscle cell phenotype and aneurysm progression. Circ Res (2009) 1.46

Fibulin-5 initiates epithelial-mesenchymal transition (EMT) and enhances EMT induced by TGF-beta in mammary epithelial cells via a MMP-dependent mechanism. Carcinogenesis (2008) 1.45

Novel MYH11 and ACTA2 mutations reveal a role for enhanced TGFβ signaling in FTAAD. Int J Cardiol (2011) 1.41

alpha(v)beta(3) Integrin interacts with the transforming growth factor beta (TGFbeta) type II receptor to potentiate the proliferative effects of TGFbeta1 in living human lung fibroblasts. J Biol Chem (2004) 1.41

Interferon-gamma and the interferon-inducible chemokine CXCL10 protect against aneurysm formation and rupture. Circulation (2009) 1.40

Angiotensin AT2 receptor contributes to cardiovascular remodelling of aged rats during chronic AT1 receptor blockade. J Mol Cell Cardiol (2004) 1.40

Altered TGFbeta signaling and cardiovascular manifestations in patients with autosomal recessive cutis laxa type I caused by fibulin-4 deficiency. Eur J Hum Genet (2010) 1.39

Absence of TGFbeta signaling in embryonic vascular smooth muscle leads to reduced lysyl oxidase expression, impaired elastogenesis, and aneurysm. Genesis (2009) 1.37

S100A12 mediates aortic wall remodeling and aortic aneurysm. Circ Res (2009) 1.36

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

Involvement of alphavbeta5 integrin-mediated activation of latent transforming growth factor beta1 in autocrine transforming growth factor beta signaling in systemic sclerosis fibroblasts. Arthritis Rheum (2005) 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

Involvement of metalloproteinases 2/9 in epidermal growth factor receptor transactivation in pressure-induced myogenic tone in mouse mesenteric resistance arteries. Circulation (2004) 1.31

Fibulin-4 conducts proper elastogenesis via interaction with cross-linking enzyme lysyl oxidase. Proc Natl Acad Sci U S A (2009) 1.30

Compound heterozygous mutations in fibulin-4 causing neonatal lethal pulmonary artery occlusion, aortic aneurysm, arachnodactyly, and mild cutis laxa. Am J Med Genet A (2007) 1.29

Integrin alphavbeta3, requirement for VEGFR2-mediated activation of SAPK2/p38 and for Hsp90-dependent phosphorylation of focal adhesion kinase in endothelial cells activated by VEGF. Cell Stress Chaperones (2003) 1.29

Losartan decreases cardiac muscle fibrosis and improves cardiac function in dystrophin-deficient mdx mice. J Cardiovasc Pharmacol Ther (2011) 1.28

Integrin-mediated transforming growth factor-beta activation, a potential therapeutic target in fibrogenic disorders. Am J Pathol (2009) 1.25

Filamin A-mediated down-regulation of the exchange factor Ras-GRF1 correlates with decreased matrix metalloproteinase-9 expression in human melanoma cells. J Biol Chem (2007) 1.23

New insights into the pathogenesis of autosomal-dominant cutis laxa with report of five ELN mutations. Hum Mutat (2011) 1.20

Fibulin-5 antagonizes vascular endothelial growth factor (VEGF) signaling and angiogenic sprouting by endothelial cells. DNA Cell Biol (2004) 1.20

Articles by these authors

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

A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet (2005) 9.62

Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med (2006) 9.50

Angiotensin II blockade and aortic-root dilation in Marfan's syndrome. N Engl J Med (2008) 7.31

The revised Ghent nosology for the Marfan syndrome. J Med Genet (2010) 6.87

Nonsense surveillance regulates expression of diverse classes of mammalian transcripts and mutes genomic noise. Nat Genet (2004) 6.14

Exosome-mediated recognition and degradation of mRNAs lacking a termination codon. Science (2002) 5.46

Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states. Nat Med (2007) 5.25

An mRNA surveillance mechanism that eliminates transcripts lacking termination codons. Science (2002) 5.22

Atenolol versus losartan in children and young adults with Marfan's syndrome. N Engl J Med (2014) 5.11

Marfan's syndrome. Lancet (2005) 4.99

TGF-beta-dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome. J Clin Invest (2004) 4.85

Separable roles for rent1/hUpf1 in altered splicing and decay of nonsense transcripts. Science (2002) 3.84

Noncanonical TGFβ signaling contributes to aortic aneurysm progression in Marfan syndrome mice. Science (2011) 3.49

Treatment of aortic disease in patients with Marfan syndrome. Circulation (2005) 3.48

Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism. Science (2011) 3.18

Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome. J Clin Invest (2004) 3.01

Lessons on the pathogenesis of aneurysm from heritable conditions. Nature (2011) 2.97

Mutations in the facilitative glucose transporter GLUT10 alter angiogenesis and cause arterial tortuosity syndrome. Nat Genet (2006) 2.94

Dissection of epistasis in oligogenic Bardet-Biedl syndrome. Nature (2005) 2.90

Circulating transforming growth factor-beta in Marfan syndrome. Circulation (2009) 2.69

Association between a functional variant of the KLOTHO gene and high-density lipoprotein cholesterol, blood pressure, stroke, and longevity. Circ Res (2005) 2.63

Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm. Nat Genet (2012) 2.44

Association of human aging with a functional variant of klotho. Proc Natl Acad Sci U S A (2002) 2.38

Mutations in the TGF-β repressor SKI cause Shprintzen-Goldberg syndrome with aortic aneurysm. Nat Genet (2012) 2.34

Perturbations of vascular homeostasis and aortic valve abnormalities in fibulin-4 deficient mice. Circ Res (2007) 2.32

Rationale and design of a randomized clinical trial of beta-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome. Am Heart J (2007) 2.14

TGFβ receptor mutations impose a strong predisposition for human allergic disease. Sci Transl Med (2013) 2.08

Control of liver cell fate decision by a gradient of TGF beta signaling modulated by Onecut transcription factors. Genes Dev (2005) 2.02

Marfan syndrome: from molecular pathogenesis to clinical treatment. Curr Opin Genet Dev (2007) 1.99

KLOTHO allele status and the risk of early-onset occult coronary artery disease. Am J Hum Genet (2003) 1.90

Integrin-modulating therapy prevents fibrosis and autoimmunity in mouse models of scleroderma. Nature (2013) 1.86

DSG2 mutations contribute to arrhythmogenic right ventricular dysplasia/cardiomyopathy. Am J Hum Genet (2006) 1.85

Early surgical experience with Loeys-Dietz: a new syndrome of aggressive thoracic aortic aneurysm disease. Ann Thorac Surg (2007) 1.75

Phenotypic spectrum of the SMAD3-related aneurysms-osteoarthritis syndrome. J Med Genet (2012) 1.62

Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in the thoracic aortic aneurysm in Marfan syndrome. Circ Res (2007) 1.61

Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg (2002) 1.59

Familial thoracic aortic dilation and bicommissural aortic valve: a prospective analysis of natural history and inheritance. Am J Med Genet A (2007) 1.54

Synergistic heterozygosity for TGFbeta1 SNPs and BMPR2 mutations modulates the age at diagnosis and penetrance of familial pulmonary arterial hypertension. Genet Med (2008) 1.49

A Pkd1-Fbn1 genetic interaction implicates TGF-β signaling in the pathogenesis of vascular complications in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2013) 1.49

Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome. J Vasc Surg (2008) 1.48

Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology. Physiol Genomics (2004) 1.45

Identification of candidate regions for familial idiopathic scoliosis. Spine (Phila Pa 1976) (2005) 1.43

Novel MYH11 and ACTA2 mutations reveal a role for enhanced TGFβ signaling in FTAAD. Int J Cardiol (2011) 1.41

Tgfbr2 disruption in postnatal smooth muscle impairs aortic wall homeostasis. J Clin Invest (2014) 1.40

Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years. Ann Thorac Surg (2009) 1.39

Altered TGFbeta signaling and cardiovascular manifestations in patients with autosomal recessive cutis laxa type I caused by fibulin-4 deficiency. Eur J Hum Genet (2010) 1.39

Allelic variation in normal human FBN1 expression in a family with Marfan syndrome: a potential modifier of phenotype? Hum Mol Genet (2003) 1.37

Aortic root operations for Marfan syndrome: a comparison of the Bentall and valve-sparing procedures. Ann Thorac Surg (2008) 1.37

Interaction between krit1 and malcavernin: implications for the pathogenesis of cerebral cavernous malformations. Neurosurgery (2007) 1.35

Recent advances in understanding Marfan syndrome: should we now treat surgical patients with losartan? J Thorac Cardiovasc Surg (2008) 1.35

Histopathologic findings in ascending aortas from individuals with Loeys-Dietz syndrome (LDS). Am J Surg Pathol (2009) 1.34

Extracellular microfibrils in vertebrate development and disease processes. J Biol Chem (2009) 1.34

Impact of image analysis methodology on diagnostic and surgical classification of patients with thoracic aortic aneurysms. Ann Thorac Surg (2011) 1.32

Angiotensin II-dependent TGF-β signaling contributes to Loeys-Dietz syndrome vascular pathogenesis. J Clin Invest (2013) 1.32

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

Growth and maturation in Marfan syndrome. Am J Med Genet (2002) 1.28

Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events. J Cell Physiol (2007) 1.27

Toward an understanding of dural ectasia: a light microscopy study in a murine model of Marfan syndrome. Spine (Phila Pa 1976) (2005) 1.27

Loeys-Dietz syndrome: MDCT angiography findings. AJR Am J Roentgenol (2007) 1.23

Nuclear mRNA degradation pathway(s) are implicated in Xist regulation and X chromosome inactivation. PLoS Genet (2006) 1.22

Induction of macrophage chemotaxis by aortic extracts of the mgR Marfan mouse model and a GxxPG-containing fibrillin-1 fragment. Circulation (2006) 1.20

Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on research in Marfan syndrome and related disorders. Circulation (2008) 1.18

The National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC): results from phase I and scientific opportunities in phase II. Am Heart J (2011) 1.17

Angiotensin receptor blockade attenuates cigarette smoke-induced lung injury and rescues lung architecture in mice. J Clin Invest (2011) 1.17

Dual functions for LTBP in lung development: LTBP-4 independently modulates elastogenesis and TGF-beta activity. J Cell Physiol (2009) 1.14

Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy. Am Heart J (2013) 1.14

Therapy of Marfan syndrome. Annu Rev Med (2008) 1.09

Progressive dilation of the ascending aorta in children with isolated bicuspid aortic valve. Am J Cardiol (2007) 1.08

Molecular genetic and biochemical analyses of FGF23 mutations in familial tumoral calcinosis. Am J Physiol Endocrinol Metab (2008) 1.05

Endothelial expression of hypoxia-inducible factor 1 protects the murine heart and aorta from pressure overload by suppression of TGF-β signaling. Proc Natl Acad Sci U S A (2012) 1.04

Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome. Hum Mol Genet (2010) 1.03

Microfibril structure masks fibrillin-2 in postnatal tissues. J Biol Chem (2010) 1.03

Perturbation of thymocyte development in nonsense-mediated decay (NMD)-deficient mice. Proc Natl Acad Sci U S A (2011) 1.03

Targeting collagen strands by photo-triggered triple-helix hybridization. Proc Natl Acad Sci U S A (2012) 1.01

Characterization of the symptoms associated with dural ectasia in the Marfan patient. Am J Med Genet A (2005) 1.00

Krit1 modulates beta 1-integrin-mediated endothelial cell proliferation. Neurosurgery (2008) 0.97

Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: Analysis of early outcome. J Thorac Cardiovasc Surg (2009) 0.97

Inhibition of nonsense-mediated mRNA decay by the natural product pateamine A through eukaryotic initiation factor 4AIII. J Biol Chem (2009) 0.96

Long-term implications of emergency versus elective proximal aortic surgery in patients with Marfan syndrome in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium Registry. J Thorac Cardiovasc Surg (2011) 0.96

Inhibition of nonsense-mediated RNA decay activates autophagy. Mol Cell Biol (2013) 0.95

Targeted disruption of NeuroD, a proneural basic helix-loop-helix factor, impairs distal lung formation and neuroendocrine morphology in the neonatal lung. J Biol Chem (2008) 0.94

Altered tissue behavior of a non-aneurysmal descending thoracic aorta in the mouse model of Marfan syndrome. Cell Tissue Res (2011) 0.93

Causes and histopathology of ascending aortic disease in children and young adults. Cardiovasc Pathol (2010) 0.93

Results of aortic valve-sparing operations: experience with remodeling and reimplantation procedures in 65 patients. Ann Thorac Surg (2004) 0.91

Bone lessons from Marfan syndrome and related disorders: fibrillin, TGF-B and BMP at the balance of too long and too short. Pediatr Endocrinol Rev (2013) 0.90

Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms. J Vasc Surg (2012) 0.90

Protrusio acetabuli in Marfan syndrome: age-related prevalence and associated hip function. J Bone Joint Surg Am (2006) 0.90

Musculoskeletal findings of Loeys-Dietz syndrome. J Bone Joint Surg Am (2010) 0.88

Valve-sparing aortic root replacement in Loeys-Dietz syndrome. Ann Thorac Surg (2011) 0.88

Association between fetal lymphedema and congenital cardiovascular defects in Turner syndrome. Pediatrics (2005) 0.87

Valve-sparing aortic root replacement in children: intermediate-term results. Interact Cardiovasc Thorac Surg (2010) 0.87

Constrained selected reaction monitoring: quantification of selected post-translational modifications and protein isoforms. Methods (2013) 0.86

Pravastatin reduces Marfan aortic dilation. Circulation (2011) 0.86

LMNA-associated cardiocutaneous progeria: an inherited autosomal dominant premature aging syndrome with late onset. Am J Med Genet A (2013) 0.85

Genotype-phenotype correlation in patients with bicuspid aortic valve and aneurysm. J Thorac Cardiovasc Surg (2012) 0.84

Mitral valve disease in Marfan syndrome and related disorders. J Cardiovasc Transl Res (2011) 0.84

Valve-sparing aortic root replacement: early experience with the De Paulis Valsalva graft in 51 patients. Ann Thorac Surg (2006) 0.83