Objective    To validate the mouse carrying Ser252Trp mutation in fibroblast growth factor receptor (FGFR2) as a model for human Apert syndrome resulting from equivalent mutation by quantitatively analyzing the morphologic changes of mouse skull.     Methods    Euclidean distance matrix analysis (EDMA) was used to quantitatively compare the morphologic difference between the skulls of mutants and their wild type

controls by analyzing the 3D coordinate data of skulls’27 landmarks collected by a 3D coordinate Measuring Machine.     Results    There was significant differences in substantial morphology between skulls of mutants and their matched controls (

P

<

0.01). The mutants showed shortened length along anterior-posterior axis

especially in the anterior portion of skulls. The mutants exhibited significantly decreased lengths in the nasal bone

premaxilla

maxilla and zygoma (

P

<

0.01)

as well as broadened interorbital and frontal bone width (

P

<

0.01). All these anomalies subsequently led to shortened A-P length

as well as the increased width and height in mutant skulls

which highly similar to those skull features of humans with Apert syndrome such as brachycephaly

midface dysplasia

widened distance between eyes

shortened nasal bones

and expanded cranial volumes

etc.

     Conclusion    The FGFR2 Ser252Trp mutation shows the characteristic skull phenotypes that resemble those of human with Apert syndrome. Our results indicate that this mouse strain can be used as a good model for human Apert syndrome.