Depletion of Mast Cells and Macrophages Impairs Heterotopic Ossification in an Acvr1^R206H Mouse Model of Fibrodysplasia Ossificans Progressiva.

ABSTRACT: Heterotopic ossification (HO) is a clinical condition that often reduces mobility and diminishes quality of life for affected individuals. The most severe form of progressive HO occurs in those with fibrodysplasia ossificans progressiva (FOP; OMIM #135100), a genetic disorder caused by a recurrent heterozygous gain-of-function mutation (R206H) in the bone morphogenetic protein (BMP) type I receptor ACVR1/ALK2. In individuals with FOP, episodes of HO frequently follow injury. The first sign of active disease is commonly an inflammatory "flare-up" that precedes connective tissue degradation, progenitor cell recruitment, and endochondral HO. We used a conditional-on global knock-in mouse model expressing Acvr1^R206H (referred to as Acvr1^cR206H/+ ) to investigate the cellu

SUBMITTER: Convente MR 

PROVIDER: S-EPMC7737844 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature