Heterotopic Ossification in Mouse Models of Fibrodysplasia Ossificans Progressiva
| Autor: | Salin A Chakkalakal, Eileen M. Shore |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Mice Transgenic 030209 endocrinology & metabolism ACVR1 Biology Cardiotoxins Article Mice 03 medical and health sciences 0302 clinical medicine medicine Animals Allele Alleles Ossification Ossification Heterotopic Genetic disorder X-Ray Microtomography medicine.disease Chondrogenesis Immunohistochemistry Disease Models Animal 030104 developmental biology Myositis Ossificans Genetically Engineered Mouse Fibrodysplasia ossificans progressiva Mutation Cancer research Heterotopic ossification medicine.symptom Activin Receptors Type I Biomarkers |
| Zdroj: | Methods in Molecular Biology ISBN: 9781493989034 Methods Mol Biol |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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