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Significance We outline the musculoskeletal phenotype in a Hutchinson–Gilford progeria syndrome (HGPS) mouse model (G608G) that accurately simulates the changes found in humans with this disease, to evaluate the effects of treatments on natural course of the disease. As cartilage abnormalities seen in this HGPS model resemble those observed in the age-matched WT controls, further studies at different growth stages would allow researchers to understand cartilage health and structure to determine whether cartilage degeneration in these mice is attributable to premature aging of the tissue or due to a developmental alteration. This raises the possibility of using these mice as a model resembling degenerative joint disease in the elderly, toward early detection, management, and development of novel therapeutics for osteoarthritis.

Evaluation of musculoskeletal phenotype of the G608G progeria mouse model with lonafarnib, pravastatin, and zoledronic acid as treatment groups