Article
Cell differentiation and matrix organization are differentially affected during bone formation in osteogenesis imperfecta zebrafish models with different genetic defects impacting collagen type I structure.
Matrix biology : journal of the International Society for Matrix Biology - 1 Aug 2023
Daponte Valentina, Tonelli Francesca, Masiero Cecilia, Syx Delfien, Exbrayat-Héritier Chloé, Biggiogera Marco, Willaert Andy, Rossi Antonio, Coucke Paul J, Ruggiero Florence, Forlino Antonella
Abstract excerpt
Osteogenesis imperfecta (OI) is a family of rare heritable skeletal disorders associated with dominant mutations in the collagen type I encoding genes and recessive defects in proteins involved in collagen type I synthesis and processing and in osteoblast differentiation and activity. Historically, it was believed that the OI bone phenotype was only caused by abnormal collagen type I fibrils in the extracellular...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
