Searchable abstracts of presentations at key conferences on calcified tissues

ba0007oc13 | (1) | ICCBH2019

Analysis of osteogenesis imperfecta in pathology and the effects of 4-phenylbutyric acid using patient-derived fibroblasts and induced pluripotent stem cells

Takeyari Shinji , Ohata Yasuhisa , Kubota Takuo , Taga Yuki , Mizuno Kazunori , Ozono Keiichi

Objectives: Osteogenesis Imperfecta (OI) is a heritable brittle bone disease mainly caused by mutation of COL1A1 or COL1A2. Treatment with bisphosphonate is not effective enough in patients with severe OI. 4-phenylbutyric acid (4-PBA) may become a new medicine, which was reported to ameliorate the phenotype of an OI zebrafish model. In the present study, we aimed to analyze the pathology of OI and the effects of 4-PBA on patient-derived fibroblasts and induced pluripotent stem...