Research ArticleGENETIC DISORDERS

Two tissue-resident progenitor lineages drive distinct phenotypes of heterotopic ossification

Science Translational Medicine  23 Nov 2016:
Vol. 8, Issue 366, pp. 366ra163
DOI: 10.1126/scitranslmed.aaf1090

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Boning up on stem cells

Fibrodysplasia ossificans progressiva is a rare genetic disorder of uncontrolled bone growth, which manifests as formation of bone in the muscles, ligaments, and joints triggered by inflammation or injury. Dey et al. used mouse models with disease-causing mutations in ACVR1 protein localized to specific types of progenitor cells in ligaments or in muscles to clarify the biology of this disease and determine which cells give rise to abnormal bone. In addition, the authors showed that the harmful manifestations of these mutations can be successfully controlled by a selective inhibitor of ACVR1.

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