(Q38215407)

31 August 2017

title

Cardiovascular manifestations in Marfan syndrome and related diseases; multiple genes causing similar phenotypes (English)

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31 August 2017

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J R Cook

1

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31 August 2017

L Carta

series ordinal

2

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31 August 2017

J Galatioto

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3

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31 August 2017

F Ramirez

series ordinal

4

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31 August 2017

publication date

10 July 2014

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31 August 2017

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31 August 2017

volume

87

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31 August 2017

issue

1

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31 August 2017

page(s)

11-20

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The Marfan Syndrome: Diagnosis and Management

31 August 2017

cites work

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21 January 2018

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Marfan syndrome: from molecular pathogenesis to clinical treatment

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Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1

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Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome

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Fibrillins 1 and 2 perform partially overlapping functions during aortic development

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A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2

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Mutations in the TGF-β repressor SKI cause Shprintzen-Goldberg syndrome with aortic aneurysm

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Mutations in SMAD3 cause a syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis

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The molecular genetics of Marfan syndrome and related disorders

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The revised Ghent nosology for the Marfan syndrome

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Color flow and conventional echocardiography of the Marfan syndrome

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Cardiovascular magnetic resonance in Marfan syndrome

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Histopathology and fibrillin-1 distribution in severe early onset Marfan syndrome

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Molecular mechanisms of thoracic aortic dissection

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Aortopathies: etiologies, genetics, differential diagnosis, prognosis and management

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The new Ghent criteria for Marfan syndrome: what do they change?

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Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome

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Acute aortic dissection: population-based incidence compared with degenerative aortic aneurysm rupture

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Valve-sparing aortic root replacement in Marfan syndrome

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Mechanisms of abdominal aortic aneurysm formation

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Medical management of Marfan syndrome

21 January 2018

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Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: an ECHOCARDIOgraphic study

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Prognosis factors in probands with an FBN1 mutation diagnosed before the age of 1 year

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Evidence for Marfan cardiomyopathy

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Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases.

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Beyond the root: dilatation of the distal aorta in Marfan's syndrome

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Is close radiographic and clinical control after repair of acute type A aortic dissection really necessary for improved long-term survival?

21 January 2018

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Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan's syndrome

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Management of Marfan syndrome

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Phenotypic features and impact of beta blocker or calcium antagonist therapy on aortic lumen size in the Marfan syndrome

21 January 2018

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Thoracic aortic aneurysm frequency and dissection are associated with fibrillin-1 fragment concentrations in circulation

21 January 2018

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Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: analysis of early outcome

21 January 2018

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Long-term results of aortic root repair using the reimplantation technique

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Fibrillin: from domain structure to supramolecular assembly

21 January 2018

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Extracellular microfibrils: contextual platforms for TGFbeta and BMP signaling

21 January 2018

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TGFβ signalling in context

21 January 2018

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Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome

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Generation of Fbn1 conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment

21 January 2018

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Vascular extracellular matrix and aortic development.

21 January 2018

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The fibrillin-1 hypomorphic mgR/mgR murine model of Marfan syndrome shows severe elastolysis in all segments of the aorta.

21 January 2018

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p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice

21 January 2018

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Neutralization of transforming growth factor-beta attenuates hypertension and prevents renal injury in uremic rats

21 January 2018

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Effects of AT1- and beta-adrenergic receptor antagonists on TGF-beta1-induced fibrosis in transgenic mice.

21 January 2018

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Noncanonical TGFβ signaling contributes to aortic aneurysm progression in Marfan syndrome mice

21 January 2018

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Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism

21 January 2018

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Angiotensin II blockade and aortic-root dilation in Marfan's syndrome

21 January 2018

1 reference

Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial

21 January 2018

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MMP-2 regulates Erk1/2 phosphorylation and aortic dilatation in Marfan syndrome

21 January 2018

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Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome

21 January 2018

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

21 January 2018

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Antagonism of GxxPG fragments ameliorates manifestations of aortic disease in Marfan syndrome mice

21 January 2018

1 reference

The Loeys-Dietz syndrome: an update for the clinician

21 January 2018

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Comparison of clinical presentations and outcomes between patients with TGFBR2 and FBN1 mutations in Marfan syndrome and related disorders.

21 January 2018

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Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm

21 January 2018

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Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome

21 January 2018

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In frame fibrillin-1 gene deletion in autosomal dominant Weill-Marchesani syndrome

21 January 2018

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Fibrillin-1 mutations causing Weill-Marchesani syndrome and acromicric and geleophysic dysplasias disrupt heparan sulfate interactions

21 January 2018

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Integrin-modulating therapy prevents fibrosis and autoimmunity in mouse models of scleroderma

21 January 2018

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Intrinsic biventricular dysfunction in Marfan syndrome.

21 January 2018

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Left ventricular diastolic dysfunction in children and young adults with Marfan syndrome

21 January 2018

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Primary impairment of left ventricular function in Marfan syndrome.

21 January 2018

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Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death?

21 January 2018

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Left ventricular function in the Marfan syndrome without significant valvular regurgitation

21 January 2018

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Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome

21 January 2018

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Effectiveness of combination of losartan potassium and doxycycline versus single-drug treatments in the secondary prevention of thoracic aortic aneurysm in Marfan syndrome

21 January 2018

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miR-29b participates in early aneurysm development in Marfan syndrome.

21 January 2018

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Phenotypic spectrum of the SMAD3-related aneurysms–osteoarthritis syndrome

21 January 2018

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Tissue Doppler imaging identifies myocardial dysfunction in adults with marfan syndrome

21 January 2018

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21 January 2018

Identifiers

DOI

10.1111/CGE.12436

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31 August 2017

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