Article
4PBA reduces growth deficiency in osteogenesis imperfecta by enhancing transition of hypertrophic chondrocytes to osteoblasts.
JCI insight - 8 Feb 2022
Scheiber Amanda L, Wilkinson Kevin J, Suzuki Akiko, Enomoto-Iwamoto Motomi, Kaito Takashi, Cheah Kathryn Se, Iwamoto Masahiro, Leikin Sergey, Otsuru Satoru
Abstract excerpt
Short stature is a major skeletal phenotype in osteogenesis imperfecta (OI), a genetic disorder mainly caused by mutations in genes encoding type I collagen. However, the underlying mechanism is poorly understood, and no effective treatment is available. In OI mice that carry a G610C mutation in COL1A2, we previously found that mature hypertrophic chondrocytes (HCs) are exposed to cell stress due to accumulation...
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