Fig. 8.

Anti‐TGF‐β antibody 1D11 restores osteoblast differentiation as evidenced by effects on runt‐related transcription factor 2 (RUNX2) and alkaline phosphatase (ALP). Immunohistochemistry performed in lumbar bone (A) shows increased RUNX2 expression on the trabecular bone surface in osteogenesis imperfecta (OI) +13C4 relative to WT + 13C4. Treatment with 1D11 corrects RUNX2 expression to WT levels at three‐times weekly dosing for 12 weeks and is dose‐frequency related. Dosing regimen: 3 × wk = three weekly doses; 1 × 2 wk = one dose every other week; 1 × 4 wk = one dose every 4 weeks; all doses 5 mg/kg ip. (B) ALP expression, both in trabecular and cortical bone is decreased in OI +13C4 relative to WT +13C4 and is partially restored with 1D11 treatment, short‐term study three‐time weekly dosing, four doses, all doses 5 mg/kg ip. n = 3, 3, and 6 for groups 1–3, respectively.