"Wang, Y." . . "Oberoi, K." . . "15"^^ . "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 . "Richtsmeier, J. T." . "p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice." . "Couwenhoven, R." . . "Huso, D. L." . "p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice." . . . "122" . . . . "0021-9738" . "000304736300023" . . "Rendl, Michael" . . "p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice."@en . . "Wang Jabs, E." . . "Zhou, X." . . "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." . . "I, P(GA301/09/0587), P(GAP305/11/0752), S, Z(MSM0021622430)" . . . "10.1172/JCI62644" . "[327E245DFF4D]" . "RIV/00216224:14310/12:00065919!RIV14-MSM-14310___" . "Friedenthal, J." . . . "RIV/00216224:14310/12:00065919" . "6" . "14310" . "p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice."@en . . . "Rezza, A." . . . "The Journal of Clinical Investigation" . "Percival, Ch. J." . "12"^^ . "p38; skin; skull; FGFR2; Beare-Stevenson syndrome"@en . "US - Spojen\u00E9 st\u00E1ty americk\u00E9" . "1"^^ . "Sun, M." . "Phelps, R." . . "Holmes, G." . "Krej\u010D\u00ED, Pavel" . "163704" .