Haibo Xie, Yunsi Kang, Junjun Liu, Min Huang, Zhicheng Dai, Jiale Shi, Shuo Wang, Lanqin Li, Yuan Li, Pengfei Zheng, Yi Sun, Qize Han, Jingjing Zhang, Zezhang Zhu, Leilei Xu, Pamela C Yelick, Muqing Cao, Chengtian Zhao
Idiopathic scoliosis (IS) is the most common spinal deformity diagnosed in childhood or early adolescence, while the underlying pathogenesis of this serious condition remains largely unknown. Here, we report zebrafish ccdc57 mutants exhibiting scoliosis during late development, similar to that observed in human adolescent idiopathic scoliosis (AIS). Zebrafish ccdc57 mutants developed hydrocephalus due to cerebrospinal fluid (CSF) flow defects caused by uncoordinated cilia beating in ependymal cells. Mechanistically, Ccdc57 localizes to ciliary basal bodies and controls the planar polarity of ependymal cells through regulating the organization of microtubule networks and proper positioning of basal bodies...
March 2, 2023: PLoS Biology