Article
Targeting cellular stress in vitro improves osteoblast homeostasis, matrix collagen content and mineralization in two murine models of osteogenesis imperfecta.
Matrix biology : journal of the International Society for Matrix Biology - 1 Apr 2021
Garibaldi Nadia, Contento Barbara M, Babini Gabriele, Morini Jacopo, Siciliani Stella, Biggiogera Marco, Raspanti Mario, Marini Joan C, Rossi Antonio, Forlino Antonella, Besio Roberta
Abstract excerpt
Most cases of dominantly inherited osteogenesis imperfecta (OI) are caused by glycine substitutions in the triple helical domain of type I collagen α chains, which delay collagen folding, and cause the synthesis of collagen triple helical molecules with abnormal structure and post-translational modification. A variable extent of mutant collagen ER retention and other secondary mutation effects perturb osteoblast...
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