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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)
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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)
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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)
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skos:notation
| - RIV/00216224:14310/12:00065919!RIV14-MSM-14310___
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http://linked.open...avai/predkladatel
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http://linked.open...avai/riv/aktivita
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http://linked.open...avai/riv/aktivity
| - I, P(GA301/09/0587), P(GAP305/11/0752), S, Z(MSM0021622430)
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http://linked.open...iv/cisloPeriodika
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http://linked.open...vai/riv/dodaniDat
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http://linked.open...aciTvurceVysledku
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http://linked.open.../riv/druhVysledku
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http://linked.open...iv/duvernostUdaju
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http://linked.open...titaPredkladatele
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http://linked.open...dnocenehoVysledku
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http://linked.open...ai/riv/idVysledku
| - RIV/00216224:14310/12:00065919
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http://linked.open...riv/jazykVysledku
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http://linked.open.../riv/klicovaSlova
| - p38; skin; skull; FGFR2; Beare-Stevenson syndrome (en)
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http://linked.open.../riv/klicoveSlovo
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http://linked.open...odStatuVydavatele
| - US - Spojené státy americké
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http://linked.open...ontrolniKodProRIV
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http://linked.open...i/riv/nazevZdroje
| - The Journal of Clinical Investigation
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http://linked.open...in/vavai/riv/obor
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http://linked.open...ichTvurcuVysledku
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http://linked.open...cetTvurcuVysledku
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http://linked.open...vavai/riv/projekt
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http://linked.open...UplatneniVysledku
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http://linked.open...v/svazekPeriodika
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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.
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http://linked.open...ain/vavai/riv/wos
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http://linked.open...n/vavai/riv/zamer
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http://bibframe.org/vocab/doi
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http://localhost/t...ganizacniJednotka
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