A Review of the Application of Zebrafish in Ciliopathy Research

WEI Zhiying^1,2^#, JIANG Shangkun³^#, CHEN Changshui⁴, LI Xinhua⁵, AN Yu^1,6*

（¹Institute of Human Phenomics, Fudan University, Shanghai201203, China; ²Department of Laboratory Medicine, Zhongshan Hospital, Fudan University, Shanghai 200032, China; ³School of Medicine, Tongji University, Shanghai 200092, China; ⁴Ningbo Key Laboratory for the Prevention and Treatment of Embryogenic Diseases, the Affiliated Women and Children’s Hospital of Ningbo University, Ningbo 315000, China; ⁵Department of Orthopedics, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200080, China; ⁶Institute of Medical Genetics, Fudan University, Shanghai 200030, China)

Abstract:

Cilia, as microtubule-based subcellular structure, widely present on the surface of nearly all vertebrate cells and play crucial roles in various biological processes such as cell motility and signal transduction. A series of genetic diseases caused by abnormalities in the structure or function of cilia are collectively referred to as ciliopathies. Due to the high evolutionary conservation of cilia and the similarity between zebrafish and humans in multiple specialized ciliary types, zebrafish has emerged as an ideal model organism for studying ciliary functions and ciliopathies. This paper summarizes the structural features of zebrafish cilia, sorts out the advances in commonly used related novel techniques, and describes the important findings and contributions of the zebrafish model in the study of human ciliopathy. Studies have shown that zebrafish, with its unique biological development characteristics and highly similar ciliary structure to that of humans, has greatly contributed to the research progress in ciliarelated fields and provided important clues for a deeper understanding of the pathogenesis of ciliopathies.

CSTR: 32200.14.cjcb.2025.07.0022