NIH R01 · 2024
Discoidin Domain Receptor 2, β1 Integrins and ECM Control of Bone Formation
Discoidin domain receptor 2 (DDR2) is a non-integrin collagen receptor having important but poorly understood skeletal functions. Inactivating mutations in DDR2 cause spondylo-meta-epiphyseal dysplasia (SMED-short limb type), a human disorder with severe craniofacial and skeletal abnormalities. Ddr2-deficient mice have a similar phenotype including abnormal skull shape, delayed suture fusion and defective cartilage growth/orientation in synchondroses at the base of the skull, shortened long bone growth plates and reduced trabecular and cortical bone mass. Preliminary studies where Ddr2 was selectively inactivated in skeletal progenitors indicate distinct functions in cells of the bone…
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