Published in Curr Opin Genet Dev on April 27, 2007
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Fibrillin-1 and -2 differentially modulate endogenous TGF-β and BMP bioavailability during bone formation. J Cell Biol (2010) 1.49
Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome. J Vasc Surg (2008) 1.48
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The role of the renin-angiotensin system in aortic aneurysmal diseases. Curr Hypertens Rep (2008) 1.28
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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
Vascular complications in autosomal dominant polycystic kidney disease. Nat Rev Nephrol (2015) 0.84
The c.7409G>A (p.Cys2470Tyr) Variant of FBN1: Phenotypic Variability across Three Generations. Mol Syndromol (2013) 0.82
Generation of Fbn1 conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment. Genesis (2012) 0.81
Valve-sparing aortic root replacement in patients with Marfan syndrome enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions. J Heart Valve Dis (2014) 0.80
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Chondroitin sulfate and growth factor signaling in the skeleton: Possible links to MPS VI. J Pediatr Rehabil Med (2010) 0.76
Corneal Deformation Response and Ocular Geometry: A Noninvasive Diagnostic Strategy in Marfan Syndrome. Am J Ophthalmol (2015) 0.76
Heart rate turbulence and deceleration capacity for risk prediction of serious arrhythmic events in Marfan syndrome. Clin Res Cardiol (2015) 0.76
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Novel pharmacological strategies to prevent aortic complications in Marfan syndrome. J Geriatr Cardiol (2011) 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
Isolated infrarenal abdominal aorta aneurysm in a 42-year-old patient with Marfan's syndrome: Case report. SAGE Open Med Case Rep (2013) 0.75
Fibrillin-1 microfibrils influence adult bone marrow hematopoiesis. Matrix Biol (2015) 0.75
An iPSC-derived vascular model of Marfan syndrome identifies key mediators of smooth muscle cell death. Nat Genet (2016) 0.75
Latent TGF-β binding protein 2 and 4 have essential overlapping functions in microfibril development. Sci Rep (2017) 0.75
Complications associated with surgical repair of syndromic scoliosis. Scoliosis (2015) 0.75
The influence of Nrf2 on cardiac responses to environmental stressors. Oxid Med Cell Longev (2013) 0.75
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Fibrillin microfibrils: structural tensometers of elastic tissues? Int J Exp Pathol (2017) 0.75
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
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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
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
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
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
Angiotensin II-dependent TGF-β signaling contributes to Loeys-Dietz syndrome vascular pathogenesis. J Clin Invest (2013) 1.32
Matrix-dependent perturbation of TGFβ signaling and disease. FEBS Lett (2012) 1.31
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
Perturbation of thymocyte development in nonsense-mediated decay (NMD)-deficient mice. Proc Natl Acad Sci U S A (2011) 1.03
Microfibril structure masks fibrillin-2 in postnatal tissues. J Biol Chem (2010) 1.03
Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome. Hum Mol Genet (2010) 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
Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms. J Vasc Surg (2012) 0.90
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
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
Mitral valve disease in Marfan syndrome and related disorders. J Cardiovasc Transl Res (2011) 0.84
Genotype-phenotype correlation in patients with bicuspid aortic valve and aneurysm. J Thorac Cardiovasc Surg (2012) 0.84
Valve-sparing aortic root replacement: early experience with the De Paulis Valsalva graft in 51 patients. Ann Thorac Surg (2006) 0.83
Murine model of surgically induced acute aortic dissection type A. J Thorac Cardiovasc Surg (2009) 0.83