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Role of altered signal transduction in heterotopic ossification and fibrodysplasia ossificans progressiva.
Curr Osteoporos Rep. 2011 Jun; 9(2):83-8.CO

Abstract

Heterotopic ossification is a pathologic condition in which bone tissue is formed outside of the skeleton, within soft tissues of the body. The extraskeletal bone that forms in these disorders is normal; the cellular mechanisms that direct cell fate decisions are dysregulated. Patients with fibrodysplasia ossificans progressiva (FOP), a rare human genetic disorder of extensive and progressive heterotopic ossification, have malformations of normal skeletal elements, identifying the causative gene mutation and its relevant signaling pathways as key regulators of skeletal development and of cell fate decisions by adult stem cells. The discovery that mildly activating mutations in ACVR1/ALK2, a bone morphogenetic protein (BMP) type I receptor, is the cause of FOP has provided opportunities to identify previously unknown functions for this receptor and for BMP signaling and to develop new diagnostic and therapeutic strategies for FOP and other more common forms of heterotopic ossification, as well as tissue engineering applications.

Authors+Show Affiliations

Department of Orthopaedic Surgery, University of Pennsylvania School of Medicine, 426 Stemmler Hall, 3450 Hamilton Walk, Philadelphia, PA 19104-6081, USA. shore@mail.med.upenn.eduNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

21340697

Citation

Shore, Eileen M., and Frederick S. Kaplan. "Role of Altered Signal Transduction in Heterotopic Ossification and Fibrodysplasia Ossificans Progressiva." Current Osteoporosis Reports, vol. 9, no. 2, 2011, pp. 83-8.
Shore EM, Kaplan FS. Role of altered signal transduction in heterotopic ossification and fibrodysplasia ossificans progressiva. Curr Osteoporos Rep. 2011;9(2):83-8.
Shore, E. M., & Kaplan, F. S. (2011). Role of altered signal transduction in heterotopic ossification and fibrodysplasia ossificans progressiva. Current Osteoporosis Reports, 9(2), 83-8. https://doi.org/10.1007/s11914-011-0046-3
Shore EM, Kaplan FS. Role of Altered Signal Transduction in Heterotopic Ossification and Fibrodysplasia Ossificans Progressiva. Curr Osteoporos Rep. 2011;9(2):83-8. PubMed PMID: 21340697.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Role of altered signal transduction in heterotopic ossification and fibrodysplasia ossificans progressiva. AU - Shore,Eileen M, AU - Kaplan,Frederick S, PY - 2011/2/23/entrez PY - 2011/2/23/pubmed PY - 2011/11/16/medline SP - 83 EP - 8 JF - Current osteoporosis reports JO - Curr Osteoporos Rep VL - 9 IS - 2 N2 - Heterotopic ossification is a pathologic condition in which bone tissue is formed outside of the skeleton, within soft tissues of the body. The extraskeletal bone that forms in these disorders is normal; the cellular mechanisms that direct cell fate decisions are dysregulated. Patients with fibrodysplasia ossificans progressiva (FOP), a rare human genetic disorder of extensive and progressive heterotopic ossification, have malformations of normal skeletal elements, identifying the causative gene mutation and its relevant signaling pathways as key regulators of skeletal development and of cell fate decisions by adult stem cells. The discovery that mildly activating mutations in ACVR1/ALK2, a bone morphogenetic protein (BMP) type I receptor, is the cause of FOP has provided opportunities to identify previously unknown functions for this receptor and for BMP signaling and to develop new diagnostic and therapeutic strategies for FOP and other more common forms of heterotopic ossification, as well as tissue engineering applications. SN - 1544-2241 UR - https://www.unboundmedicine.com/medline/citation/21340697/Role_of_altered_signal_transduction_in_heterotopic_ossification_and_fibrodysplasia_ossificans_progressiva_ L2 - https://dx.doi.org/10.1007/s11914-011-0046-3 DB - PRIME DP - Unbound Medicine ER -