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

Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F68081707%3A_____%2F12%3A00390407" target="_blank" >RIV/68081707:_____/12:00390407 - isvavai.cz</a>
Alternative codes found
RIV/00216224:14310/12:00065919
Result on the web
<a href="http://dx.doi.org/10.1172/JCI62644" target="_blank" >http://dx.doi.org/10.1172/JCI62644</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1172/JCI62644" target="_blank" >10.1172/JCI62644</a>

Result language
angličtina
Original language name
p38 Inhibition ameliorates skin and skull abnormalities in Fgfr2 Beare-Stevenson mice
Original language 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.
Czech name
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Czech description
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Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
BO - Biophysics
OECD FORD branch
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Project
Result was created during the realization of more than one project. More information in the Projects tab.
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year
2012
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

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