Ontology highlight
ABSTRACT:
SUBMITTER: Lees-Shepard JB
PROVIDER: S-EPMC6143342 | biostudies-literature | 2018 Sep
REPOSITORIES: biostudies-literature
Lees-Shepard John B JB Nicholas Sarah-Anne E SE Stoessel Sean J SJ Devarakonda Parvathi M PM Schneider Michael J MJ Yamamoto Masakazu M Goldhamer David J DJ
eLife 20180918
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by debilitating heterotopic ossification (HO). The retinoic acid receptor gamma agonist, palovarotene, and antibody-mediated activin A blockade have entered human clinical trials, but how these therapeutic modalities affect the behavior of pathogenic fibro/adipogenic progenitors (FAPs) is unclear. Using live-animal luminescence imaging, we show that transplanted pathogenic FAPs undergo rapid initial expansion, w ...[more]