Molecular mechanisms of regulation in locomotive tissues

我们研究了骨形态发生蛋白(BMP)信号诱导C2C12成肌细胞异位成骨的分子机制。Smad依赖的途径在诱导成骨细胞分化和抑制肌肉生成方面起着关键作用。依赖Smad的信号被Zranb2或Dragon抑制。在进行性骨化性纤维发育不良(FOP)患者中，BMP受体之一ALK2发生了基因突变，激活了Smad依赖的信号转导，而与BMPs的结合无关。

We studied molecular mechanisms of heterotopic bone formation induced by bone morphogenetic protein(BMP) signaling in C2C12 myoblasts. The Smad-dependent pathway plays critical roles for both an induction of osteoblast differentiation and inhibition of myogenesis. The Smad-dependent signaling was suppressed by Zranb2 or DRAGON. In patients with fibrodysplasia ossificans progressiva(FOP), one of BMP receptors, ALK2, was genetically mutated and activated the Smad-dependent signaling regardless of the binding to BMPs.