LaBonty Melissa, Yelick Pamela C
Program in Cell, Molecular, and Developmental Biology, Sackler School of Graduate Biomedical Sciences, Tufts University School of Medicine, Boston, Massachusetts.
Department of Orthodontics, Division of Craniofacial and Molecular Genetics, Tufts University School of Dental Medicine, Boston, Massachusetts.
Dev Dyn. 2018 Feb;247(2):279-288. doi: 10.1002/dvdy.24606. Epub 2017 Dec 1.
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.
进行性骨化性纤维发育不良是一种罕见的异位骨化人类疾病。FOP患者在纤维组织内经历异位骨的进行性发展,这会导致活动能力逐渐丧失,并可能导致早期死亡。由于对人类FOP的病因和进展缺乏了解,而且手术干预往往会加剧FOP疾病的进展,因此需要包括动物模型在内的替代治疗方法,以研究和增进对人类FOP的理解。在这篇综述中,我们概述了现有的FOP动物模型以及每个模型的关键机制研究结果。此外,我们强调了我们实验室生成的一种新的成年斑马鱼模型在研究人类FOP方面的特定优势。《发育动力学》247:279 - 288,2018年。©2017威利期刊公司。