Characterization of the craniofacial abnormalities of the homozygous G608G progeria mouse model.

INTRODUCTION

Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by premature aging, impacting multiple organ systems, including cardiovascular, musculoskeletal, and integumentary. Significant abnormalities in a transgenic mouse model (homozygous G608G mutation), specifically targeting the development of skull and facial bone indices through high-resolution CT scanning and cephalometric analysis.

METHODS

Key measurements include bone thickness, skull volume, and cranial suture integrity. Bone volume increased significantly in HGPS mice by 8 months of age compared to wildtype mice.

RESULTS

Cortical thickness showed a trend toward increased values in HGPS mice. Cranial metrics revealed distinct differences.

DISCUSSION: HGPS mice exhibited smaller internasal width, interzygomatic distance, and palatine length compared to WT mice over time.