Cbfa1-deficient mice were found to show a complete lack of bone formation owing to the maturational arrest of osteoblasts. Cbfa1 plays key roles in the determination of osteoblastic lineage from multipotential mesenchymal cells, their differentiation into mature osteoblasts, and transcriptional regulation of bone matrix-related genes Cbfa1 positively regulates chondrocyte maturation and osteoclast differentiation and is required for vascular invasion into cartilage. Therefore, complete elucidation of the function of Cbfa1 and its signaling would be of great benefit in understanding skeletogenesis.