Abstract
Genetic Disorder Fibrodysplasia ossificans progressiva (FOP) is a rare but deadly genetic condition that causes bone growth in place of soft tissues. The causal mutation in the bone morphogenetic protein (BMP) receptor ACVR1 is thought to boost receptor activity and trigger bone formation. Now, Harsell et al. suggest a different mechanism. They found that the mutated receptor (mACVR1) responds to activin. Normally, activin blocks BMP from binding to ACVR1. Adult mice expressing mACVR1 developed heterotopic ossification that required stimulation by endogenous activin. Small sponges soaked with activin ossified when implanted into mACVR1 mice, and animals treated with a monoclonal antibody to activin were protected. This unusual mechanism may explain why ossification in FOP patients is triggered by tissue trauma, which induces activin. Sci. Transl. Med. 7 , 303ra137 (2015).
Published Version
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