Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disorder in which injury or trauma to fibrous tissues, such as muscle and connective tissue, causes these soft tissues to turn to bone. Ossification of tissues leads to joint fusion and breathing impairment owing to restriction of the thoracic cavity. Since there is no known cure, this disease causes devastating disability and a shortened life span. Patients with this disease harbor a mutation in the bone morphogenic protein (BMP) type 1 receptor ALK2 that probably leads to constitutively active kinase activity. Therefore, Yu et al. studied transgenic mice with inducible, constitutive ALK2 activity in order to investigate FOP in a mouse model. They found that ALK2 induction led to tissue ossification and that chemical inhibition of ALK2 kinase activity reduced this pathogenic process. This study supports the role of ALK2 kinase activity in FOP and suggests that BMP signaling mediation might be a potential therapy for this disease.
Connective tissue disorders: kinase causes progressive ossification in soft tissue
Connective tissue disorders: kinase causes progressive ossification in soft tissue. Dis Model Mech 7 January 2009; 2 (1-2): 2. doi:
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