title: Zebrafish as a Model for Human Ciliopathies.
abstract: Cilia, microtubule-based structures found on the surface of almost all vertebrate cells, play an array of diverse biological functions. Abnormal ciliary axonemal structure and function can result in a class of genetic disorders that are collectively termed ciliopathies. Model organisms, including Chlamydomonas reinhardtii and Caenorhabditis elegans have been widely used to study the complex genetic basis of ciliopathies. Here, we review the advantages of the zebrafish as a vertebrate model for human ciliopathies. We summarize the features of zebrafish cilia, and the major findings and contributions of the zebrafish model in recent studies of human ciliopathies. We also discuss the new genome editing approaches being efficiently used in zebrafish, and the exciting prospects of these approaches in modeling human ciliopathies.
authors.affiliation: Institute of Evolution & Marine Biodiversity, College of Marine Life Science, Ocean University of China, Qingdao 266003, China.
authors.forename: Zhu
authors.initials: Z
authors.lastname: Song
authors.affiliation: Institute of Evolution & Marine Biodiversity, College of Marine Life Science, Ocean University of China, Qingdao 266003, China.
authors.forename: Xiaoli
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Institute of Evolution & Marine Biodiversity, College of Marine Life Science, Ocean University of China, Qingdao 266003, China.
authors.forename: Shuo
authors.initials: S
authors.lastname: Jia
authors.affiliation: Department of Orthodontics, Division of Craniofacial and Molecular Genetics, Tufts University, Boston, MA 02111, USA.
authors.forename: Pamela C
authors.initials: PC
authors.lastname: Yelick
authors.affiliation: Institute of Evolution & Marine Biodiversity, College of Marine Life Science, Ocean University of China, Qingdao 266003, China; Laboratory for Marine Biology and Biotechnology, Qingdao National Laboratory for Marine Science and Technology, Qingdao 266003, China. Electronic address: chengtian_zhao@ouc.edu.cn.
authors.forename: Chengtian
authors.initials: C
authors.lastname: Zhao
mesh_terms: D000818:Animals; D002923:Cilia / Q000378:metabolism / Q000473:pathology; D000072661:Ciliopathies / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D004195:Disease Models, Animal; D006801:Humans; D015027:Zebrafish
keywords: Cilia; Ciliopathy; Disease model; Zebrafish