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Heterotopic Ossification in Mouse Models of Fibrodysplasia Ossificans Progressiva.
- Source :
-
Methods in molecular biology (Clifton, N.J.) [Methods Mol Biol] 2019; Vol. 1891, pp. 247-255. - Publication Year :
- 2019
-
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 <superscript>R206H</superscript> , 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 <superscript>R206H</superscript> allele.
- Subjects :
- Activin Receptors, Type I genetics
Alleles
Animals
Biomarkers
Cardiotoxins adverse effects
Disease Models, Animal
Immunohistochemistry
Mice
Mice, Transgenic
Mutation
Myositis Ossificans diagnostic imaging
Myositis Ossificans etiology
Ossification, Heterotopic diagnostic imaging
Ossification, Heterotopic etiology
X-Ray Microtomography
Myositis Ossificans pathology
Ossification, Heterotopic pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1940-6029
- Volume :
- 1891
- Database :
- MEDLINE
- Journal :
- Methods in molecular biology (Clifton, N.J.)
- Publication Type :
- Academic Journal
- Accession number :
- 30414138
- Full Text :
- https://doi.org/10.1007/978-1-4939-8904-1_18