For instance, when the TGF-β signaling pathway and modulators were targeted (with 1D11 antibody and soluble ActRIIB), different responses were reported in the oim, Amish, Crtap and Jrt OI murine models [107,117], due to their underlying bone phenotype, which in turn is caused by different mutations leading to different collagen type I defects with distinct severity. This evidence concerns the gene ACVR2B and osteogenesis imperfecta.