Transgenic Model Of Fibrodisplasia Ossificans Progressiva (FOP)
- Technology Benefits
- Closelyapproximates human FOP disease
- Detailed Technology Description
- New mouse model of FOP, a rare genetic disease characterized by abnormal growth of bone which replaces soft tissues #researchtool #animalmodel
- *Abstract
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Northwestern University scientists developed a mouse model of fibrodisplasia ossificans progressiva (FOP), a rare genetic disease that causes soft tissues to be replaced with bone over time. While it was understood that bone morphogenetic protein-4 (BMP-4) was involved in the pathophysiology of this disease, this knowledge did not facilitate the generation of a good mouse model of FOP for many years. Northwestern researchers were able to generate a transgenic mouse model that most closely mimics human disease by overexpressing BMP-4 under the control of the neuron specific enolase (NSE) promoter. This model was used to confirm the role of BMP-4 in FOP and could enable further research that may lead to the development of a treatment for this disabling disease.
- *Inventors
- John A. Kessler
- *Publications
- Kan L, Hu M, Gomes WA,Kessler JA (2004). Transgenic Mice Overexpressing BMP4 Develop a FibrodisplasiaOssificans Progressiva (FOP)-Like Phenotype, American Journal of Pathology. 165(4): 1107-1115.
- Country/Region
- USA
