Published in Spine (Phila Pa 1976) on February 01, 2005
Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science (2006) 10.25
Marfan's syndrome. Lancet (2005) 4.99
Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome. Sci Transl Med (2010) 2.55
ADAMTSL2 mutations in geleophysic dysplasia demonstrate a role for ADAMTS-like proteins in TGF-beta bioavailability regulation. Nat Genet (2008) 1.78
Long-term effects of losartan on structure and function of the thoracic aorta in a mouse model of Marfan syndrome. Br J Pharmacol (2009) 0.82
Adhesion to the extracellular matrix is required for interleukin-1 beta actions leading to reactive phenotype in rat astrocytes. Mol Cell Neurosci (2010) 0.81
A Case of Neonatal Marfan Syndrome: A Management Conundrum and the Role of a Multidisciplinary Team. Case Rep Pediatr (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
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
Autograft-derived spinal cord mass following olfactory mucosal cell transplantation in a spinal cord injury patient: Case report. J Neurosurg Spine (2014) 3.38
Mechanisms of disease: molecular genetics of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Nat Clin Pract Cardiovasc Med (2008) 3.19
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
Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation (2005) 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
Clinical features of arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in plakophilin-2. Circulation (2006) 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
Complications of growing-rod treatment for early-onset scoliosis: analysis of one hundred and forty patients. J Bone Joint Surg Am (2010) 2.46
Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm. Nat Genet (2012) 2.44
Morphologic variants of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy a genetics-magnetic resonance imaging correlation study. J Am Coll Cardiol (2009) 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
Distinguishing arrhythmogenic right ventricular cardiomyopathy/dysplasia-associated mutations from background genetic noise. J Am Coll Cardiol (2011) 2.31
Comprehensive desmosome mutation analysis in north americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Cardiovasc Genet (2009) 2.19
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
Adolescent idiopathic scoliosis: modern management guidelines. J Surg Orthop Adv (2006) 2.11
Antifibrinolytic agents reduce blood loss during pediatric vertebral column resection procedures. Spine (Phila Pa 1976) (2012) 2.09
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
Studying arrhythmogenic right ventricular dysplasia with patient-specific iPSCs. Nature (2013) 2.00
Marfan syndrome: from molecular pathogenesis to clinical treatment. Curr Opin Genet Dev (2007) 1.99
Clinical Presentation, Long-Term Follow-Up, and Outcomes of 1001 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Patients and Family Members. Circ Cardiovasc Genet (2015) 1.94
KLOTHO allele status and the risk of early-onset occult coronary artery disease. Am J Hum Genet (2003) 1.90
Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain (2005) 1.87
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
Nedd4 controls animal growth by regulating IGF-1 signaling. Sci Signal (2008) 1.67
Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. J Am Coll Cardiol (2013) 1.66
Phenotypic spectrum of the SMAD3-related aneurysms-osteoarthritis syndrome. J Med Genet (2012) 1.62
Failed exstrophy closure: management and outcome. J Pediatr Urol (2009) 1.62
Missense mutations in plakophilin-2 cause sodium current deficit and associate with a Brugada syndrome phenotype. Circulation (2013) 1.61
Low profile pelvic fixation: anatomic parameters for sacral alar-iliac fixation versus traditional iliac fixation. Spine (Phila Pa 1976) (2009) 1.60
Use of a coronary sinus lead and biventricular ICD to correct a sensing abnormality in a patient with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Cardiovasc Electrophysiol (2006) 1.59
Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg (2002) 1.59
Incremental value of cardiac magnetic resonance imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. J Am Coll Cardiol (2013) 1.58
Factors associated with use of bone morphogenetic protein during pediatric spinal fusion surgery: an analysis of 4817 patients. J Bone Joint Surg Am (2013) 1.58
Familial thoracic aortic dilation and bicommissural aortic valve: a prospective analysis of natural history and inheritance. Am J Med Genet A (2007) 1.54
Phospholamban R14del mutation in patients diagnosed with dilated cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy: evidence supporting the concept of arrhythmogenic cardiomyopathy. Eur J Heart Fail (2012) 1.51
Plasma BIN1 correlates with heart failure and predicts arrhythmia in patients with arrhythmogenic right ventricular cardiomyopathy. Heart Rhythm (2012) 1.49
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
The application of pelvic osteotomy in adult female patients with exstrophy: applications and outcomes. BJU Int (2011) 1.47
Pelvic fixation in spine surgery. Historical overview, indications, biomechanical relevance, and current techniques. J Bone Joint Surg Am (2005) 1.47
Penetrance of mutations in plakophilin-2 among families with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol (2006) 1.46
Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology. Physiol Genomics (2004) 1.45
Prevalence of atrial arrhythmias in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Heart Rhythm (2013) 1.45
Identification of candidate regions for familial idiopathic scoliosis. Spine (Phila Pa 1976) (2005) 1.43
Growing rods for spinal deformity: characterizing consensus and variation in current use. J Pediatr Orthop (2010) 1.42
Evaluation of pelvic floor muscular redistribution after primary closure of classic bladder exstrophy by 3-dimensional magnetic resonance imaging. J Urol (2012) 1.41
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
Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol (2010) 1.39
Spica casting for pediatric femoral fractures: a prospective, randomized controlled study of single-leg versus double-leg spica casts. J Bone Joint Surg Am (2012) 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