进行性骨化性纤维增殖不良症（FOP，也称进行性骨化性肌炎）的病因是骨形态发生蛋白（BMP）Ⅰ型受体之一ACVR1发生了R206H突变，该突变导致了ACVR1(R206H)对配体的结合力和/或反应性发生了明显变化。软组织炎症或创伤是FOP发作的诱因，但FOP的致病因子的种类不清楚（activin A可能是其中之一）。我们发现在发作期FOP病人血单个核细胞中BMP6 mRNA含量明显高于正常人，提示BMP家族配体可能参与了致病。我们将用已建立的人FOP模型细胞、模型小鼠和FOP患者生物样本，研究与ACVR1（R206H）结合的配体种类及其致病作用。该研究可为针对致病因子的综合性防治提供理论基础，也为受体学理论库增加新知识。

The etiology of fibrodysplasia ossificans progressiva(FOP) is the R206H mutation in ACVR1 which is one of the receptors of bone morphogenetic protein(BMP), This mutation causes great changes in the binding ability to ligand and/or responsiveness to ligand of ACVR1(R206H).The inflammation in soft tissues or trauma is the inducer of FOP flare-up, but the nature of disease-causing factors is not clear（activin A may be one of them）. We have found that the BMP6 mRNA content was significantly higher than that of normal controls, suggesting that the BMP family ligands may participate in the pathogenesis of FOP. Based on our estabolished human FOP model cells, FOP model mice and the biological samples of FOP patients, we will investigate what the ligands are for ACVR1(R206H), and whether these ligands could be causative factors. The results may provide basis for integrated FOP therapy targeting on causative factors, and may be a valuable addition to the receptor theory.