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Heterotopic Ossification in Mouse Models of Fibrodysplasia Ossificans Progressiva.
Methods Mol Biol. 2019; 1891:247-255.MM

Abstract

Fibrodysplasia ossificans progressiva (FOP), a rare genetic disorder of progressive extra-skeletal ossification, is the most disabling form of heterotopic ossification (HO) in humans. Most people with FOP carry an activating mutation in a BMP type I receptor gene, ACVR1 R206H, that promotes ectopic chondrogenesis and osteogenesis and in turn HO. Advances in elucidating the cellular and molecular events and mechanisms that lead to the ectopic bone formation are being made through the use of genetically engineered mouse models that recapitulate the human disease. We describe methods for inducing heterotopic ossification in a mouse model that conditionally expresses the Acvr1 R206H allele.

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Publisher Full Text (DOI)

Authors+Show Affiliations

Chakkalakal SA
Department of Orthopaedic Surgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA. Center for Research in FOP and Related Disorders, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
Shore EM
Department of Orthopaedic Surgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA. shore@pennmedicine.upenn.edu. Center for Research in FOP and Related Disorders, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA. shore@pennmedicine.upenn.edu. Department of Genetics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA. shore@pennmedicine.upenn.edu.

MeSH

Activin Receptors, Type IAllelesAnimalsBiomarkersCardiotoxinsDisease Models, AnimalImmunohistochemistryMiceMice, TransgenicMutationMyositis OssificansOssification, HeterotopicX-Ray Microtomography

Pub Type(s)

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

Language

eng

PubMed ID

30414138

Citation

Chakkalakal, Salin A., and Eileen M. Shore. "Heterotopic Ossification in Mouse Models of Fibrodysplasia Ossificans Progressiva." Methods in Molecular Biology (Clifton, N.J.), vol. 1891, 2019, pp. 247-255.
Chakkalakal SA, Shore EM. Heterotopic Ossification in Mouse Models of Fibrodysplasia Ossificans Progressiva. Methods Mol Biol. 2019;1891:247-255.
Chakkalakal, S. A., & Shore, E. M. (2019). Heterotopic Ossification in Mouse Models of Fibrodysplasia Ossificans Progressiva. Methods in Molecular Biology (Clifton, N.J.), 1891, 247-255. https://doi.org/10.1007/978-1-4939-8904-1_18
Chakkalakal SA, Shore EM. Heterotopic Ossification in Mouse Models of Fibrodysplasia Ossificans Progressiva. Methods Mol Biol. 2019;1891:247-255. PubMed PMID: 30414138.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Heterotopic Ossification in Mouse Models of Fibrodysplasia Ossificans Progressiva. AU - Chakkalakal,Salin A, AU - Shore,Eileen M, PY - 2018/11/11/entrez PY - 2018/11/11/pubmed PY - 2019/6/5/medline KW - ACVR1 KW - ALK2 KW - FOP KW - Fibrodysplasia ossificans progressiva KW - HO KW - Heterotopic ossification KW - Mouse model SP - 247 EP - 255 JF - Methods in molecular biology (Clifton, N.J.) JO - Methods Mol Biol VL - 1891 N2 - Fibrodysplasia ossificans progressiva (FOP), a rare genetic disorder of progressive extra-skeletal ossification, is the most disabling form of heterotopic ossification (HO) in humans. Most people with FOP carry an activating mutation in a BMP type I receptor gene, ACVR1 R206H, that promotes ectopic chondrogenesis and osteogenesis and in turn HO. Advances in elucidating the cellular and molecular events and mechanisms that lead to the ectopic bone formation are being made through the use of genetically engineered mouse models that recapitulate the human disease. We describe methods for inducing heterotopic ossification in a mouse model that conditionally expresses the Acvr1 R206H allele. SN - 1940-6029 UR - https://www.unboundmedicine.com/medline/citation/30414138/Heterotopic_Ossification_in_Mouse_Models_of_Fibrodysplasia_Ossificans_Progressiva_ DB - PRIME DP - Unbound Medicine ER -