AUTHOR=Beeram Indeevar , Cubria Maria Belen , Kamalapathy Pramod , Yeritsyan Diana , Dubose Amanda J. , Razavi Ahmad Hedayatzadeh , Nafisi Nazanin , Erdos Michael R. , Snyder Brian D. , Cabral Wayne A. , Collins Francis S. , Nazarian Ara 

TITLE=Characterization of the craniofacial abnormalities of the homozygous G608G progeria mouse model

JOURNAL=Frontiers in Physiology

VOLUME=Volume 15 - 2024

YEAR=2024

URL=https://www.frontiersin.org/journals/physiology/articles/10.3389/fphys.2024.1481985

DOI=10.3389/fphys.2024.1481985

ISSN=1664-042X

ABSTRACT=IntroductionHutchinson-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.MethodsKey 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.ResultsCortical thickness showed a trend toward increased values in HGPS mice. Cranial metrics revealed distinct differences.DiscussionHGPS mice exhibited smaller internasal width, interzygomatic distance, and palatine length compared to WT mice over time.