Article
Pulmonary and diaphragmatic pathology in collagen type I α1 mutant mice with osteogenesis imperfecta.
Pediatric research - 1 Jun 2018
Baglole Carolyn J, Liang Feng, Traboulsi Hussein, Rico de Souza Angela, Giordano Christian, Tauer Josephine T, Rauch Frank, Petrof Basil J
Abstract excerpt
BackgroundOsteogenesis imperfecta (OI) is most often caused by mutations in type I collagen genes. Respiratory complications have been largely attributed to spine and ribcage deformities. We hypothesized that direct involvement of the pulmonary parenchyma and/or diaphragm by the disease may occur.MethodsIn Col1a1Jrt/+ mice, a model of severe dominant OI, mean linear intercept length (Lm) was used to assess the...
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