Analysis of bone metabolism based on mutant ALK2 receptor assosiated with FOP.

2014 Fiscal Year Final Research Report

• Project/Area Number: 25861339
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Orthopaedic surgery
• Research Institution: Saitama Medical University
• Principal Investigator: MIYAMOTO Arei 埼玉医科大学, 医学部, 研究員 (70634591)
• Project Period (FY): 2013-04-01 – 2015-03-31
• Keywords: 骨系統疾患 / FOP / BMP / ALK2

Outline of Final Research Achievements

Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary disease, which is characterized by postnatal progressive heterotopic ossification in skeletal muscle through endochondral ossification. ALK2(R206H), a gain-of-function mutant of BMP type I receptor ALK2, have been found in patients with FOP, activates downstream signaling and induces heterotopic ossification. We have generated transgenic mice, in which ALK2(R206H) is expressed under the control of Cre/LoxP system. In the present study, we established a new model of chondrogenesis in vitro using the skeletal muscle cells prepared from ALK2(R206H) Tg mice. In conclusion, we established a new model of chondrogenesis using skeletal muscle cells. This in vitro model may be a useful tool to clarify the pathological mechanism of FOP and to develop a new treatment for FOP.

Free Research Field

骨疾患