Fibrodysplasia Ossificans Progressiva as a Candidate for Muscle Stem Cell Disease
| Project/Area Number |
23592176
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Orthopaedic surgery
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| Research Institution | National Center for Geriatrics and Gerontology |
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Principal Investigator |
HASHIMOTO Naohiro 独立行政法人国立長寿医療研究センター, 研究所再生再建医学研究部, 部長 (00208456)
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| Project Period (FY) |
2011 – 2013
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| Project Status |
Completed (Fiscal Year 2013)
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| Budget Amount *help |
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2013: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2012: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2011: ¥2,860,000 (Direct Cost: ¥2,200,000、Indirect Cost: ¥660,000)
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| Keywords | 幹細胞 / 組織・細胞 / 再生医学 / 発生・分化 / 遺伝子 / 筋幹細胞 / 筋再生 / 細胞分化 / 筋疾患 |
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| Research Abstract |
Fibrodysplasia ossificans progressiva (FOP) is a dominant inherited disease caused by a mutated receptor for bone morphogenetic proteins ALK2/ACVR1 bearing a single amino acid substitution of Arg to His at codon 206 (R206H). We found that ALK2(R206H) is a constitutively leaky but not a functionally active receptor because it stimulated Smad pathway very slightly without ligand binding, and could not induce osteogenesis in mouse myogenic progenitor cells. An inflammatory cytokine IL-6 triggered osteogenesis in myogenic cells expressing ALK2(R206H). The present results suggest that ALK2(R206H)-expressing myogenic progenitor cells act as osteoprogenitor cells when they are stimulated with IL-6 and provide a new mechanistic insight in inflammatory stimulus-triggered heterotopic bone formation in FOP patients. FOP is a candidate for a disease caused by muscle stem cells that are misled down a heterotopic osteogenic differentiation pathway.
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Report
(4 results)
Research Products
(54 results)
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[Journal Article] Cdk4 and cyclin D1 allow human myogenic cells to recapture growth property without compromising differentiation potentialπGene2011
Author(s)
Shiomi, K., Kiyono , T., Okamura, K., Uezumi, M., Goto, Y., Yasumoto, S., Shimizu, S. and Hashimoto, N
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Journal Title
Therapy
Volume: 18
Pages: 857-866
Related Report
Peer Reviewed
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[Journal Article] Cdk4 and cyclin D1 allow human myogenic cells to recapture growth property without compromising differentiation potential2011
Author(s)
Shiomi, K., Kiyono , T., Okamura, K., Uezumi, M., Goto, Y., Yasumoto, S., Shimizu, S. and Hashimoto, N
-
Journal Title
Gene Therapy
Volume: 18
Pages: 857-866
Related Report
Peer Reviewed
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