Frontiers in Physiology (Nov 2024)

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

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

DOI
https://doi.org/10.3389/fphys.2024.1481985
Journal volume & issue
Vol. 15

Abstract

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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.

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