Early onset of craniosynostosis in an Apert mouse model reveals critical features of this pathology
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A Genetic-Pathophysiological Framework for CraniosynostosisFibroblast growth factor signaling in skeletal development and diseaseDevelopmental and Evolutionary Significance of the Zygomatic BoneChanges in biomechanical strain and morphology of rat calvarial sutures and bone after Tgf-β3 inhibition of posterior interfrontal suture fusionActivation of p38 MAPK pathway in the skull abnormalities of Apert syndrome Fgfr2(+P253R) mice.Morphological comparison of the craniofacial phenotypes of mouse models expressing the Apert FGFR2 S252W mutation in neural crest- or mesoderm-derived tissues.Therapeutic effect of nanogel-based delivery of soluble FGFR2 with S252W mutation on craniosynostosis.Beyond the closed suture in apert syndrome mouse models: evidence of primary effects of FGFR2 signaling on facial shape at birth.The Sox2 high mobility group transcription factor inhibits mature osteoblast function in transgenic miceJagged1 functions downstream of Twist1 in the specification of the coronal suture and the formation of a boundary between osteogenic and non-osteogenic cellsInhibited Wnt signaling causes age-dependent abnormalities in the bone matrix mineralization in the Apert syndrome FGFR2(S252W/+) miceRegulation of cranial morphogenesis and cell fate at the neural crest-mesoderm boundary by engrailed 1.p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice.Gain-of-Function Mutations in ZIC1 Are Associated with Coronal Craniosynostosis and Learning Disability.The effect of a Beare-Stevenson syndrome Fgfr2 Y394C mutation on early craniofacial bone volume and relative bone mineral density in mice.Further analysis of the Crouzon mouse: effects of the FGFR2(C342Y) mutation are cranial bone-dependentFrom shape to cells: mouse models reveal mechanisms altering palate development in Apert syndromeAugmentation of Smad-dependent BMP signaling in neural crest cells causes craniosynostosis in mice.Mesodermal expression of Fgfr2S252W is necessary and sufficient to induce craniosynostosis in a mouse model of Apert syndrome.Understanding craniosynostosis as a growth disorderEmbryonic craniofacial bone volume and bone mineral density in Fgfr2(+/P253R) and nonmutant mice.The role of vertebrate models in understanding craniosynostosis.A familial case of Muenke syndrome. Diverse expressivity of the FGFR3 Pro252Arg mutation--case report and review of the literature.The molecular and cellular basis of Apert syndrome.Inducible activation of FGFR2 in adult mice promotes bone formation after bone marrow ablation.Apert syndrome mutant FGFR2 and its soluble form reciprocally alter osteogenesis of primary calvarial osteoblasts.A novel ciliopathic skull defect arising from excess neural crestScalp fibroblasts have a shared expression profile in monogenic craniosynostosis.FGFR2c-mediated ERK-MAPK activity regulates coronal suture development.Effects of thyroxine exposure on the Twist 1 +/- phenotype: A test of gene-environment interaction modeling for craniosynostosisWnt/β-catenin signaling and Msx1 promote outgrowth of the maxillary prominences.Craniosynostosis-associated Fgfr2(C342Y) mutant bone marrow stromal cells exhibit cell autonomous abnormalities in osteoblast differentiation and bone formation.FGFR2 mutation confers a less drastic gain of function in mesenchymal stem cells than in fibroblasts.Craniosynostosis of coronal suture in twist1 mice occurs through endochondral ossification recapitulating the physiological closure of posterior frontal suture.Mouse models of Apert syndrome.Algorithm to assess cranial suture fusion with varying and discontinuous mineral density.Soluble form of FGFR2 with S252W partially prevents craniosynostosis of the apert mouse model.The Fgfr2(S252W/+) mutation in mice retards mandible formation and reduces bone mass as in human Apert syndrome.Dynamic morphological changes in the skulls of mice mimicking human Apert syndrome resulting from gain-of-function mutation of FGFR2 (P253R).Craniosynostosis
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P2860
Early onset of craniosynostosis in an Apert mouse model reveals critical features of this pathology
description
2009 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2009 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 2009
@ast
im April 2009 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2009/04/15)
@sk
vědecký článek publikovaný v roce 2009
@cs
wetenschappelijk artikel (gepubliceerd op 2009/04/15)
@nl
наукова стаття, опублікована у квітні 2009
@uk
مقالة علمية (نشرت في 15-4-2009)
@ar
name
Early onset of craniosynostosi ...... cal features of this pathology
@ast
Early onset of craniosynostosi ...... cal features of this pathology
@en
Early onset of craniosynostosi ...... cal features of this pathology
@nl
type
label
Early onset of craniosynostosi ...... cal features of this pathology
@ast
Early onset of craniosynostosi ...... cal features of this pathology
@en
Early onset of craniosynostosi ...... cal features of this pathology
@nl
prefLabel
Early onset of craniosynostosi ...... cal features of this pathology
@ast
Early onset of craniosynostosi ...... cal features of this pathology
@en
Early onset of craniosynostosi ...... cal features of this pathology
@nl
P2093
P2860
P921
P1476
Early onset of craniosynostosi ...... cal features of this pathology
@en
P2093
Chu-Xia Deng
Claudio Basilico
Gerson Rothschild
Greg Holmes
Upal Basu Roy
P2860
P304
P356
10.1016/J.YDBIO.2009.01.026
P407
P577
2009-04-15T00:00:00Z