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The role of Activin A in fibrodysplasia ossificans progressiva: a prominent mediator.
Biosci Rep. 2019 08 30; 39(8)BR

Abstract

Heterotopic ossification (HO) is the aberrant formation of mature, lamellar bone in nonosseous tissue. Fibrodysplasia ossificans progressiva (FOP) is a rare and devastating genetic disorder that causes progressive HO in the ligaments, tendons, and muscles throughout the body. FOP is attributed to an autosomal mutation in activin receptor-like kinase 2 (ALK2), a bone morphogenetic protein (BMP) type I receptor. Initial studies show that mutant ALK2 drives HO by constitutively activating the BMP signaling pathway. Recently, mutant ALK2 has been shown to transduce Smad1/5 signaling and enhance chondrogenesis, calcification in response to Activin A, which normally signals through Smad2/3 and inhibits BMP signaling pathway. Furthermore, Activin A induces heterotopic bone formation via mutant ALK2, while inhibition of Activin A blocks spontaneous and trauma-induced HO. In this manuscript, we describe the molecular mechanism of the causative gene ALK2 in FOP, mainly focusing on the prominent role of Activin A in HO. It reveals a potential strategy for prevention and treatment of FOP by inhibition of Activin A. Further studies are needed to explore the cellular and molecular mechanisms of Activin A in FOP in more detail.

Authors+Show Affiliations

Jiangxi Province Key Laboratory of Tumor Pathogens and Molecular Pathology and Department of Pathophysiology, School of Basic Medicine Sciences, Nanchang University Medical College, Nanchang, China huilin88@ncu.edu.cn.Jiangxi Province Key Laboratory of Tumor Pathogens and Molecular Pathology and Department of Pathophysiology, School of Basic Medicine Sciences, Nanchang University Medical College, Nanchang, China.Jiangxi Province Key Laboratory of Tumor Pathogens and Molecular Pathology and Department of Pathophysiology, School of Basic Medicine Sciences, Nanchang University Medical College, Nanchang, China.Jiangxi Province Key Laboratory of Tumor Pathogens and Molecular Pathology and Department of Pathophysiology, School of Basic Medicine Sciences, Nanchang University Medical College, Nanchang, China.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

31341010

Citation

Lin, Hui, et al. "The Role of Activin a in Fibrodysplasia Ossificans Progressiva: a Prominent Mediator." Bioscience Reports, vol. 39, no. 8, 2019.
Lin H, Shi F, Gao J, et al. The role of Activin A in fibrodysplasia ossificans progressiva: a prominent mediator. Biosci Rep. 2019;39(8).
Lin, H., Shi, F., Gao, J., & Hua, P. (2019). The role of Activin A in fibrodysplasia ossificans progressiva: a prominent mediator. Bioscience Reports, 39(8). https://doi.org/10.1042/BSR20190377
Lin H, et al. The Role of Activin a in Fibrodysplasia Ossificans Progressiva: a Prominent Mediator. Biosci Rep. 2019 08 30;39(8) PubMed PMID: 31341010.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The role of Activin A in fibrodysplasia ossificans progressiva: a prominent mediator. AU - Lin,Hui, AU - Shi,Fuli, AU - Gao,Jiayu, AU - Hua,Ping, Y1 - 2019/08/02/ PY - 2019/02/15/received PY - 2019/07/16/revised PY - 2019/07/23/accepted PY - 2019/7/26/pubmed PY - 2020/9/15/medline PY - 2019/7/26/entrez KW - ALK2 KW - Activin A KW - FOP KW - Heterotopic ossification JF - Bioscience reports JO - Biosci. Rep. VL - 39 IS - 8 N2 - Heterotopic ossification (HO) is the aberrant formation of mature, lamellar bone in nonosseous tissue. Fibrodysplasia ossificans progressiva (FOP) is a rare and devastating genetic disorder that causes progressive HO in the ligaments, tendons, and muscles throughout the body. FOP is attributed to an autosomal mutation in activin receptor-like kinase 2 (ALK2), a bone morphogenetic protein (BMP) type I receptor. Initial studies show that mutant ALK2 drives HO by constitutively activating the BMP signaling pathway. Recently, mutant ALK2 has been shown to transduce Smad1/5 signaling and enhance chondrogenesis, calcification in response to Activin A, which normally signals through Smad2/3 and inhibits BMP signaling pathway. Furthermore, Activin A induces heterotopic bone formation via mutant ALK2, while inhibition of Activin A blocks spontaneous and trauma-induced HO. In this manuscript, we describe the molecular mechanism of the causative gene ALK2 in FOP, mainly focusing on the prominent role of Activin A in HO. It reveals a potential strategy for prevention and treatment of FOP by inhibition of Activin A. Further studies are needed to explore the cellular and molecular mechanisms of Activin A in FOP in more detail. SN - 1573-4935 UR - https://www.unboundmedicine.com/medline/citation/31341010/The_role_of_Activin_A_in_fibrodysplasia_ossificans_progressiva:_a_prominent_mediator_ L2 - https://portlandpress.com/bioscirep/article-lookup/doi/10.1042/BSR20190377 DB - PRIME DP - Unbound Medicine ER -
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