The Role of PEDF in Bone Mineralization
نویسنده
چکیده
BACKGROUND: Osteogenesis imperfecta (OI) type VI is a heritable disease that leads to a large number of fractures in children. It is caused by genetic mutations that lead to the absence of pigmentepithelium derived factor (PEDF) in all body cells. PEDF is a protein that is produced and secreted by cells in many tissues (liver, eye, fat). In bone, it is particularly highly expressed in osteoblasts, the bone-forming cells. The function of PEDF in these cells is not known at present. It is clear, however, that PEDF deficiency results in OI type VI.
منابع مشابه
Pigment epithelium-derived factor (PEDF) normalizes matrix defects in iPSCs derived from Osteogenesis imperfecta Type VI
Osteogenesis imperfecta (OI) Type VI is characterized by a defect in bone mineralization, which results in multiple fractures early in life. Null mutations in the PEDF gene, Serpinf1, are the cause of OI VI. Whether PEDF restoration in a murine model of OI Type VI could improve bone mass and function was previously unknown. In Belinsky et al, we provided evidence that PEDF delivery enhanced bon...
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