Article
Respiratory defects in the CrtapKO mouse model of osteogenesis imperfecta.
American journal of physiology. Lung cellular and molecular physiology - 1 Apr 2020
Dimori Milena, Heard-Lipsmeyer Melissa E, Byrum Stephanie D, Mackintosh Samuel G, Kurten Richard C, Carroll John L, Morello Roy
Abstract excerpt
Respiratory disease is a leading cause of mortality in patients with osteogenesis imperfecta (OI), a connective tissue disease that causes severely reduced bone mass and is most commonly caused by dominant mutations in type I collagen genes. Previous studies proposed that impaired respiratory function in OI patients was secondary to skeletal deformities; however, recent evidence suggests the existence of a...
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