Abstract
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by congenital malformation of the great toes and by progressive, postnatal beterotopic bone formation. Although the genetic defect in FOP is not known, several lines of evidence suggest that dysregulation of bone morphogenetic protein (BMP) 4 may be involved in the pathophysiology of the condition. Transgenic mice that overex press BMP4 under the control of the neuron-specific enolase (Nse) promoter is the first mouse model to develop progressive, postnatal heterotopic endochondral ossification. The Nse-BMP4 transgenic mouse provides a unique opportunity to study the pathophysiology and treatment of progressive heterotopic ossification in an animal model relevant to the study of FOP.
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Kan, L. The neuron-specific enolase-bone morphogenic protein 4 transgenic mouse. Clinic Rev Bone Miner Metab 3, 235–237 (2005). https://doi.org/10.1385/BMM:3:3-4:235
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DOI: https://doi.org/10.1385/BMM:3:3-4:235