【摘 要】
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Aims Fibrodysplasia ossicans progressiva (FOP;OMIM ＃135100) is a rare and severely disabling genetic disease characterized by progressive and cumulative heterotopic ossi R206H) in activin receptor typ
【机 构】
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Dept.of Endocrinology,Tongji Hospital Affiliated to Tongji University
【出 处】
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中华医学会第七次骨质疏松和骨矿盐疾病中青年学术会议
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Aims Fibrodysplasia ossicans progressiva (FOP;OMIM ＃135100) is a rare and severely disabling genetic disease characterized by progressive and cumulative heterotopic ossi R206H) in activin receptor type ⅠA (ACVR1,also known as activin-like kinase 2,ALK2),a subtype of bone morphogenetic protein (BMP) type Ⅰ receptor,contributes to both excessive Smad1/5/8and p38 MAPK signaling and then leads to extra bone formation in connective tissues.The aim of this study was to evaluate the efficacy of a potential therapeutic agent,ACVR1-Fc fusion protein,for inhibiting the function of overactive receptor ACVR1 invitro.
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