Osteogenesis of Crouzon-Mutated Cells in an Experimental Model.

Abstract

Crouzon syndrome is an autosomal-dominant congenital disease due to a mutation in the fibroblast growth factor receptor 2 protein. The purpose of this study is to evaluate wound-healing potential of Crouzon osteoblasts and adipose-derived stem cells (ADSCs) in a murine model. Parietal skull defects were created in Crouzon and mature wild-type (WT) CD-1 mice. One group of WT and Crouzon mice were left untreated. Another group was transplanted with both WT and Crouzon adipose-derived stem cells. Additional groups compared the use of a fibrin glue scaffold and periosteum removal. Skulls were harvested from each group and evaluated histologically at 8-week and/or 16-week periods. Mean areas of defect were quantified and compared via ANOVA F-test. The average area of defect after 8 and 16 weeks in untreated Crouzon mice was 15.37 ± 1.08 cm and 16.69 ± 1.51 cm, respectively. The average area of the defect in untreated WT mice after 8 and 16 weeks averaged 14.17 ± 1.88 cm and 14.96 ± 2.26 cm, respectively. WT mice with autologous ADSCs yielded an average area of 15.35 ± 1.34 cm after 16 weeks while Crouzon mice with WT ADSCs healed to an average size of 12.98 ± 1.89 cm. Crouzon ADSCs transplanted into WT mice yielded an average area of 15.47 ± 1.29 cm while autologous Crouzon ADSCs yielded an area of 14.22 ± 3.32 cm. ANOVA F-test yielded P = .415. The fibroblast growth factor receptor 2 mutation in Crouzon syndrome does not promote reossification of critical-sized defects in mature WT and Crouzon mice. Furthermore, Crouzon ADSCs do not possess osteogenic advantage over WT ADSCs.

• Authors: Andre Alcon, Philipp Metzler, Jacob Eswarakumar, Alexander T Wilson, Derek M Steinbacher
• Journal: The Journal of craniofacial surgery (J Craniofac Surg) Vol. 29 Issue 1 Pg. 237-242 (Jan 2018) ISSN: 1536-3732 [Electronic] United States
• PMID: 29065044 (Publication Type: Journal Article)

Chemical References

• Fibrin Tissue Adhesive
• Fgfr2 protein, mouse
• Receptor, Fibroblast Growth Factor, Type 2

Topics

• Adipose Tissue (cytology)
• Animals
• Cells, Cultured
• Craniofacial Dysostosis (genetics, therapy)
• Disease Models, Animal
• Fibrin Tissue Adhesive
• Mice
• Osteoblasts (physiology)
• Osteogenesis (genetics)
• Parietal Bone (injuries, physiology)
• Receptor, Fibroblast Growth Factor, Type 2 (genetics)
• Stem Cell Transplantation
• Stem Cells (physiology)
• Wound Healing (genetics)