Library Subscription: Guest
Home Begell Digital Library eBooks Journals References & Proceedings Research Collections
Critical Reviews™ in Eukaryotic Gene Expression

Impact factor: 2.385

ISSN Print: 1045-4403
ISSN Online: 2162-6502

Critical Reviews™ in Eukaryotic Gene Expression

DOI: 10.1615/CritRevEukarGeneExpr.v20.i4.20
pages 295-311

FGF Signaling in Craniofacial Biological Control and Pathological Craniofacial Development

Nan E. Hatch
Department of Orthodontics and Pediatric Dentistry, University of Michigan, USA

ABSTRACT

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.