Animal models of fibrodysplasia ossificans progressiva. Issue 2 (1st December 2017)

• Record Type: Journal Article
• Authors: LaBonty, Melissa; Yelick, Pamela C.
• Is Part Of: Developmental dynamics. Volume 247:Issue 2(2018)
• Page Start: 279
• Page End: 288
• Publication Date: 2017-12-01
• Subjects: rare disease -- Zebrafish disease model -- genetic models; Morphogenesis -- Periodicals; Anatomy -- Periodicals; Anatomie -- Périodiques; Biologie du développement -- Périodiques; 571.833
• DOI: 10.1002/dvdy.24606
• Languages: English
• ISSNs: 1058-8388

Abstract

Abstract : Fibrodysplasia Ossificans Progressiva is a rare human disease of heterotopic ossification. FOP patients experience progressive development of ectopic bone within fibrous tissues that contributes to a gradual loss of mobility and can lead to early mortality. Due to lack of understanding of the etiology and progression of human FOP, and the fact that surgical interventions often exacerbate FOP disease progression, alternative therapeutic methods are needed, including modeling in animals, to study and improve understanding of human FOP. In this review we provide an overview of the existing animal models of FOP and the key mechanistic findings from each. In addition, we highlight the specific advantages of a new adult zebrafish model, generated by our lab, to study human FOP. Developmental Dynamics 247:279–288, 2018 . © 2017 Wiley Periodicals, Inc. Key Findings: FOP is a disease characterized by explosive heterotopic ossification. FOP is caused by activating mutations in ACVR1. Roles for Activin ligand signaling in FOP have recently been discovered. Zebrafish are useful models to study human FOP.