Zebrafish as a Model for Human Ciliopathies  

作　　者：Zhu Song Xiaoli Zhang Shuo Jia Pamela C.Yelick Chengtian Zhao 

机构地区：[1]Institute of Evolution & Marine Biodiversity, College of Marine Life Science, Ocean University of China [2]Department of Orthodontics, Division of Craniofacial and Molecular Genetics, Tufts University [3]Laboratory for Marine Biology and Biotechnology, Qingdao National Laboratory for Marine Science and Technology

出　　处：《Journal of Genetics and Genomics》2016年第3期107-120,共14页遗传学报（英文版）

基　　金：supported by the grants from the National Natural Science Foundation of China (Nos. 31372274 and 31422051);Shandong Provincial Natural Science Foundation, China (No. JQ201506) to C.Z.;supported by the grant from NIH/NIDCR (R01DE018043) to P.C.Y.

摘　　要：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.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.

关 键 词：CILIA ZEBRAFISH CILIOPATHY Disease model 

分 类 号：R596[医药卫生—内科学] R-332[医药卫生—临床医学]