ba0004oc10 | (1) | ICCBH2015
Fratzl-Zelman Nadja
, Schmidt Ingo
, Roschger Paul
, Roschger Andreas
, Reich Adi
, Glorieux Francis H.
, Wagermaier Wolfgang
, Fratzl Peter
, Klaushofer Klaus
, Marini Joan C.
, Rauch Frank
Background and methods: Pigment epithelium-derived factor (PEDF) is a potent antiangiogenic factor, ubiquitously expressed and secreted in human tissues. Hypertrophic cartilage and osteoblasts express PEDF that binds to type I collagen and glycosaminoglycans in the extracellular matrix. Two rare forms of osteogenesis imperfecta (OI) with intact collagen synthesis are associated with PEDF deficiency. Histological observations revealed excessive osteoid formation and prolonged m...