Rescue of a cherubism bone marrow stromal culture phenotype by reducing TGF β signaling

We utilized a bone marrow stromal culture system to investigate changes in TGF β signaling in a mouse model for cherubism (Sh3bp2KI/KI). Interestingly, bone marrow cultures derived from cherubism mice not only displayed impaired osteoblast differentiation, but also had spontaneous osteoclast formation. PAI1, a target gene of TGFβ signaling, was elevated 2-fold in cherubism C D11b−,CD45− cells compared to wild type cells, while the expression of BAMBI, an inhibitor of TGFβ signaling, was down-regulated.

Source: Bone - March 9, 2018 Category: Orthopaedics Authors: Yaling Liu, Tulika Sharma, I-Ping Chen, Ernst Reichenberger, Yasuyoshi Ueki, Yumna Arif, Daniel Parisi, Peter Maye Tags: Full Length Article Source Type: research