Article
4-Phenylbutyric acid enhances the mineralization of osteogenesis imperfecta iPSC-derived osteoblasts.
The Journal of biological chemistry - 1 Jan 2000
Takeyari Shinji, Kubota Takuo, Ohata Yasuhisa, Fujiwara Makoto, Kitaoka Taichi, Taga Yuki, Mizuno Kazunori, Ozono Keiichi
Abstract excerpt
Osteogenesis imperfecta (OI) is a heritable brittle bone disease mainly caused by mutations in the two type I collagen genes. Collagen synthesis is a complex process including trimer formation, glycosylation, secretion, extracellular matrix (ECM) formation, and mineralization. Using OI patient-derived fibroblasts and induced pluripotent stem cells (iPSCs), we investigated the effect of 4-phenylbutyric acid...
Topics
- Calcification, Physiologic
- Cell Differentiation
- Child, Preschool
- Collagen Type I
- Endoplasmic Reticulum
- Fibroblasts
- Humans
- Induced Pluripotent Stem Cells
- Mutation
- Osteoblasts
- Osteogenesis Imperfecta
- Phenylbutyrates
- Protein Folding