Article
Galunisertib downregulates mutant type I collagen expression and promotes MSCs osteogenesis in pediatric osteogenesis imperfecta.
Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie - 1 Jun 2024
Infante Arantza, Alcorta-Sevillano Natividad, Macías Iratxe, Cabodevilla Leire, Medhat Dalia, Lafaver Brittany, Crawford Tara K, Phillips Charlotte L, Bueno Ana M, Sagastizabal Belén, Arroyo Maitane, Campino Ainara, Gerovska Daniela, Araúzo-Bravo Marcos, Gener Blanca, Rodríguez Clara I
Abstract excerpt
Qualitative alterations in type I collagen due to pathogenic variants in the COL1A1 or COL1A2 genes, result in moderate and severe Osteogenesis Imperfecta (OI), a rare disease characterized by bone fragility. The TGF-β signaling pathway is overactive in OI patients and certain OI mouse models, and inhibition of TGF-β through anti-TGF-β monoclonal antibody therapy in phase I clinical trials in OI adults is...
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