About: p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice.

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About: p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice. Goto Sponge NotDistinct Permalink

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rdf:type	skos:Concept http://linked.opendata.cz/ontology/domain/vavai/Vysledek
Description	Beare-Stevenson cutis gyrata syndrome (BSS) is a human genetic disorder characterized by skin and skull abnormalities. BSS is caused by mutations in the FGF receptor 2 (FGFR2), but the molecular mechanisms that induce skin and skull abnormalities are unclear. We developed a mouse model of BSS harboring a FGFR2 Y394C mutation and identified p38 MAPK as an important signaling pathway mediating these abnormalities. Fgfr2+/Y394C mice exhibited epidermal hyperplasia and premature closure of cranial sutures (craniosynostosis) due to abnormal cell proliferation and differentiation. We found ligand-independent phosphorylation of FGFR2 and activation of p38 signaling in mutant skin and calvarial tissues. Treating Fgfr2+/Y394C mice with a p38 kinase inhibitor attenuated skin abnormalities by reversing cell proliferation and differentiation to near normal levels. Beare-Stevenson cutis gyrata syndrome (BSS) is a human genetic disorder characterized by skin and skull abnormalities. BSS is caused by mutations in the FGF receptor 2 (FGFR2), but the molecular mechanisms that induce skin and skull abnormalities are unclear. We developed a mouse model of BSS harboring a FGFR2 Y394C mutation and identified p38 MAPK as an important signaling pathway mediating these abnormalities. Fgfr2+/Y394C mice exhibited epidermal hyperplasia and premature closure of cranial sutures (craniosynostosis) due to abnormal cell proliferation and differentiation. We found ligand-independent phosphorylation of FGFR2 and activation of p38 signaling in mutant skin and calvarial tissues. Treating Fgfr2+/Y394C mice with a p38 kinase inhibitor attenuated skin abnormalities by reversing cell proliferation and differentiation to near normal levels. (en)
Title	p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice. p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice. (en)
skos:prefLabel	p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice. p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice. (en)
skos:notation	RIV/00216224:14310/12:00065919!RIV14-MSM-14310___
http://linked.open...avai/riv/aktivita	I P S Z
http://linked.open...avai/riv/aktivity	I, P(GA301/09/0587), P(GAP305/11/0752), S, Z(MSM0021622430)
http://linked.open...iv/cisloPeriodika	6
http://linked.open...vai/riv/dodaniDat	2014
http://linked.open...aciTvurceVysledku	Krejčí, Pavel
http://linked.open.../riv/druhVysledku	J - Článek v odborném periodiku
http://linked.open...iv/duvernostUdaju	S - Úplné a pravdivé údaje nepodléhající ochraně podle zvláštních právních předpisů
http://linked.open...titaPredkladatele	Masarykova univerzita / Přírodovědecká fakulta
http://linked.open...dnocenehoVysledku	163704
http://linked.open...ai/riv/idVysledku	RIV/00216224:14310/12:00065919
http://linked.open...riv/jazykVysledku	eng - angličtina
http://linked.open.../riv/klicovaSlova	p38; skin; skull; FGFR2; Beare-Stevenson syndrome (en)
http://linked.open.../riv/klicoveSlovo	p38 skin Beare-Stevenson syndrome FGFR2 skull
http://linked.open...odStatuVydavatele	US - Spojené státy americké
http://linked.open...ontrolniKodProRIV	[327E245DFF4D]
http://linked.open...i/riv/nazevZdroje	The Journal of Clinical Investigation
http://linked.open...in/vavai/riv/obor	ED
http://linked.open...ichTvurcuVysledku	1 (xsd:int)
http://linked.open...cetTvurcuVysledku	15 (xsd:int)
http://linked.open...vavai/riv/projekt	Novel pathways of FGFR3 signaling in achondroplasia Molecular basis of FGFR3 signal transduction in skeletal dysplasia
http://linked.open...UplatneniVysledku	2012
http://linked.open...v/svazekPeriodika	122
http://linked.open...iv/tvurceVysledku	Krejčí, Pavel Wang, Y. Zhou, X. Sun, M. Couwenhoven, R. Friedenthal, J. Holmes, G. Huso, D. L. Oberoi, K. Percival, Ch. J. Phelps, R. Rendl, Michael Rezza, A. Richtsmeier, J. T. Wang Jabs, E.
http://linked.open...ain/vavai/riv/wos	000304736300023
http://linked.open...n/vavai/riv/zamer	Functional and molecular characteristics of cancer and normal stem cells - identification of targets for novel therapeutics and therapeutic strategies
issn	0021-9738
number of pages	12 (xsd:int)
http://bibframe.org/vocab/doi	10.1172/JCI62644
http://localhost/t...ganizacniJednotka	14310

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