RT Journal Article
ID 099e89c2626b1db7
A1 Hatch, Nan E.
T1 FGF Signaling in Craniofacial Biological Control and Pathological Craniofacial Development
JF Critical Reviews™ in Eukaryotic Gene Expression
JO CRE
YR 2010
FD 2011-03-09
VO 20
IS 4
SP 295
OP 311
K1 craniofacial development
K1 craniosynostosis
K1 heparin sulfate proteoglycan
K1 Frs2
K1 PLCgamma
K1 MAPK
AB Fibroblast growth factor receptors comprise a family of four evolutionarily conserved transmembrane proteins (FGFR1, FGFR2, FGFR3 and FGFR4) known to be critical for the normal development of multiple organ systems. In this review we will primarily focus upon the role of FGF/FGFR signaling as it influences the development of the craniofacial skeleton. Signaling by FGF receptors is regulated by the tissue-specific expression of FGFR isoforms, receptor subtype specific fibroblast growth factors and heparin sulfate proteoglycans. Signaling can also be limited by the expression of endogenous inhibitors. Gain-of-function mutations in FGFRs are associated with a series of congenital abnormality syndromes referred to as the craniosynostosis syndromes. Craniosynostosis is the clinical condition of premature cranial bone fusion and patients who carry craniosynostosis syndrome-associated mutations in FGFRs commonly have abnormalities of the skull vault in the form of craniosynostosis. Patients may also have abnormalities in the facial skeleton, vertebrae and digits. In this review we will discuss recent in vitro and in vivo studies investigating biologic mechanisms by which signaling through FGFRs influences skeletal development and can lead to craniosynostosis.
PB Begell House
LK https://www.dl.begellhouse.com/journals/6dbf508d3b17c437,236270b917b07d7f,099e89c2626b1db7.html