(Q48194218)

9 February 2018

title

An iPSC-derived vascular model of Marfan syndrome identifies key mediators of smooth muscle cell death (English)

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9 February 2018

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Sanjay Sinha

7

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William G Bernard

object named as

William George Bernard

3

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author name string

Alessandra Granata

1

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9 February 2018

Felipe Serrano

2

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9 February 2018

Madeline McNamara

4

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9 February 2018

Lucinda Low

5

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9 February 2018

Priya Sastry

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9 February 2018

language of work or name

English

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publication date

28 November 2016

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9 February 2018

published in

Nature Genetics

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9 February 2018

volume

49

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9 February 2018

issue

1

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9 February 2018

page(s)

97-109

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9 February 2018

The Marfan Syndrome: Diagnosis and Management

cites work

1 reference

Recent progress towards a molecular understanding of Marfan syndrome

21 January 2018

1 reference

The molecular genetics of Marfan syndrome and related disorders

21 January 2018

1 reference

Treatment of aortic disease in patients with Marfan syndrome

21 January 2018

1 reference

Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene

21 January 2018

1 reference

Targetting of the gene encoding fibrillin-1 recapitulates the vascular aspect of Marfan syndrome.

21 January 2018

1 reference

Extracellular control of TGFbeta signalling in vascular development and disease

21 January 2018

1 reference

Marfan syndrome: from molecular pathogenesis to clinical treatment

21 January 2018

1 reference

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

21 January 2018

1 reference

Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome

21 January 2018

1 reference

Transforming growth factor-beta signaling in thoracic aortic aneurysm development: a paradox in pathogenesis

21 January 2018

1 reference

Matrix-dependent perturbation of TGFβ signaling and disease

21 January 2018

1 reference

Dimorphic effects of transforming growth factor-β signaling during aortic aneurysm progression in mice suggest a combinatorial therapy for Marfan syndrome

21 January 2018

1 reference

Atenolol versus losartan in children and young adults with Marfan's syndrome

21 January 2018

1 reference

Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1

21 January 2018

1 reference

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

21 January 2018

1 reference

Induction of pluripotent stem cells from adult human fibroblasts by defined factors

21 January 2018

1 reference

Directed differentiation of embryonic origin-specific vascular smooth muscle subtypes from human pluripotent stem cells.

21 January 2018

1 reference

Cysteine substitutions in epidermal growth factor-like domains of fibrillin-1: distinct effects on biochemical and clinical phenotypes

21 January 2018

1 reference

Effect of mutation type and location on clinical outcome in 1,013 probands with Marfan syndrome or related phenotypes and FBN1 mutations: an international study

21 January 2018

1 reference

FBN1 mutation screening of patients with Marfan syndrome and related disorders: detection of 46 novel FBN1 mutations.

21 January 2018

1 reference

Generation of human vascular smooth muscle subtypes provides insight into embryological origin-dependent disease susceptibility

21 January 2018

1 reference

Early cell fate decisions of human embryonic stem cells and mouse epiblast stem cells are controlled by the same signalling pathways

21 January 2018

1 reference

Molecular regulation of vascular smooth muscle cell differentiation in development and disease

21 January 2018

1 reference

Dysfunction of endothelial and smooth muscle cells in small arteries of a mouse model of Marfan syndrome

21 January 2018

1 reference

Medical management of Marfan syndrome

21 January 2018

1 reference

Vascular smooth muscle cell phenotypic changes in patients with Marfan syndrome.

21 January 2018

1 reference

Skeletogenic phenotype of human Marfan embryonic stem cells faithfully phenocopied by patient-specific induced-pluripotent stem cells

21 January 2018

1 reference

Developmental basis of vascular smooth muscle diversity

21 January 2018

1 reference

Embryonic origins of human vascular smooth muscle cells: implications for in vitro modeling and clinical application

21 January 2018

1 reference

TGF-beta signaling in vascular biology and dysfunction

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

Marfan Sartan: a randomized, double-blind, placebo-controlled trial

21 January 2018

1 reference

Vascular smooth muscle cell hypertrophy vs. hyperplasia. Autocrine transforming growth factor-beta 1 expression determines growth response to angiotensin II.

21 January 2018

1 reference

Thrombospondin 1 mediates angiotensin II induction of TGF-beta activation by cardiac and renal cells under both high and low glucose conditions

21 January 2018

1 reference

TGF-beta activity protects against inflammatory aortic aneurysm progression and complications in angiotensin II-infused mice

21 January 2018

1 reference

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

21 January 2018

1 reference

Induction of p53 by GKLF is essential for inhibition of proliferation of vascular smooth muscle cells

21 January 2018

1 reference

p53, p21(WAF1/CIP1), and MDM2 involvement in the proliferation and apoptosis in an in vitro model of conditionally immortalized human vascular smooth muscle cells

21 January 2018

1 reference

MEK5 is activated by shear stress, activates ERK5 and induces KLF4 to modulate TNF responses in human dermal microvascular endothelial cells

21 January 2018

1 reference

Regulation of cellular functions by the ERK5 signalling pathway

21 January 2018

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Mechanotransduction and extracellular matrix homeostasis

21 January 2018

1 reference

Cell biology. Dysfunctional mechanosensing in aneurysms

21 January 2018

1 reference

Fibrillin-integrin interactions in health and disease

21 January 2018

1 reference

Mechanical stretch-induced apoptosis in smooth muscle cells is mediated by beta1-integrin signaling pathways

21 January 2018

1 reference

Human Splicing Finder: an online bioinformatics tool to predict splicing signals

21 January 2018

1 reference

Derivation of pluripotent epiblast stem cells from mammalian embryos

21 January 2018

1 reference

Generation of functional hepatocytes from human embryonic stem cells under chemically defined conditions that recapitulate liver development

21 January 2018

1 reference

The cardiovascular aspects of Marfan's syndrome: a heritable disorder of connective tissue

21 January 2018

1 reference

12 December 2020

Marfan syndrome decreases Ca2+ wave frequency and vasoconstriction in murine mesenteric resistance arteries without changing underlying mechanisms

1 reference

12 December 2020

Signal transduction mechanisms mediating the physiological and pathophysiological actions of angiotensin II in vascular smooth muscle cells.

1 reference

12 December 2020

Fibrillin controls TGF-beta activation

1 reference

12 December 2020

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

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12 December 2020

Cell surface-localized matrix metalloproteinase-9 proteolytically activates TGF-beta and promotes tumor invasion and angiogenesis

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12 December 2020

Abnormal muscle mechanosignaling triggers cardiomyopathy in mice with Marfan syndrome.

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12 December 2020

Transforming growth factor-beta induction of type-1 plasminogen activator inhibitor. Pericellular deposition and sensitivity to exogenous urokinase

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12 December 2020

Pericellular plasmin induces smooth muscle cell anoikis.

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12 December 2020

Identifiers

DOI

10.1038/NG.3723

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9 February 2018

release_barjlelc6rfehfewh7lijruvla

Fatcat ID

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Fatcat

https://api.fatcat.wiki/v0/release/barjlelc6rfehfewh7lijruvla

24 November 2022

mapped directly with Wikidata item

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9 February 2018