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Transgenic Model Of Fibrodisplasia Ossificans Progressiva (FOP)

技術優勢
Closelyapproximates human FOP disease
詳細技術說明
New mouse model of FOP, a rare genetic disease characterized by abnormal growth of bone which replaces soft tissues #researchtool #animalmodel
*Abstract

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.
國家/地區
美國

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