Membrane covered spots in the calvaria where 3 or more bones converge. They're larger than sutures at birth but rapidly diminish in size as calvarial bones grow
What is palatal expansion using distraction osteogenesis?
Force is applied using an expansion screw which can separate the 2 halves of the maxillary complex. Tension induced bone growth & deposition at the intermaxillary/palatine suture results in maxillary expansion
What is Apert Syndrome and what does it look like?
Mutation in FGFR2 receptor which results in midface malformations including malocclusion of teeth, delayed eruption, unusual palatal morphologies (narrow palate, lateral swellings of gum, cleft soft palate, bifid uvula etc.)
during bone development at sutures, FGF is a growth factor that causes the undifferentiated mesenchymal cells to become osteoprogenitor cells. How does this occur?
via gene twist which leads to differentiation/Pre-osteoblast development
how were the mice with craniosynostosis used to model and understand the disorder?
a drug was used to inhibits FGF receptor activation and it showed the normal situation where the parietal and frontal bones go back to being 2 independent bones with an intervening coronal suture.
- this showed that the over activation of the FGF receptor has caused the craniosynostosis and that the drug that inhibited the FGF receptor prevented the sutures from fusing.
FGF receptors are mutated so they are activated with or without ligand (depending on the type of mutation) and this leads to too much twist gene and too much bone formation which leads to closure of sutures.
-HYPOTHESIS (this is what we think is happening after doing research using mice)