| [ Summary ] |
It is now clear that fibroblast growth factor receptor 3 is a major factor for the regulation of the cell proliferation and differentiation in cartilage, because of its localization and the relation between the mutations and the chondrodysplasia. But the molecular details in the pathway from the mutation through the phenotype are largely unclear. Constitutively activated mutation of FGFR-3 triggers intracellular signals such as, tyrosine phosphorylation and activation of stat1, following cellular differentiation and regulation of the other chondrocytic regulatory factors, PTHrP and Ihh. These sequential molecular events may lead to the specific phenotype. However, the mechanism of Muenke's craniosynosotosis and the mechanism of the difference between thanatophoric dysplasia and SADAAN have not been clarified so far. Moreover, natural ligand for FGFR-3 or the principal ligand involved in the pathogenetic pathway of the chondrodysplasia has not been clarified. The development in the research on these problems may lead us to understand of the molecular physiology of the cartilage and the molecular mechanisms of the growth. |