Cardiac health, type I collagen, and aging in the mouse model of osteogenesis imperfecta and a cohort of adults with OI.

Osteogenesis imperfecta (OI) is a heritable connective tissue disorder with marked skeletal fragility and increased recognition as a pleiotropic type I collagenopathy. The impact of OI-causing gene variants on cardiac health and lifespan is just beginning to be understood. To begin to investigate cardiac manifestations of OI-causing type I collagen variants, we utilized the osteogenesis imperfecta murine () model to examine survival with increased age, as well as cardiac function and collagen expression at 4 and 18 mo of age.