Primary cilia in hard tissue development and diseases  

作　　者：Sijin Li Han Zhang Yao Sun 

机构地区：[1]Department of Orthodontics,School&Hospital of Stomatology,Tongji University,Shanghai Engineering Research Center of Tooth Restoration and Regeneration,Shanghai 200072,China [2]Department of Implantology,School&Hospital of Stomatology,Tongji University,Shanghai Engineering Research Center of Tooth Restoration and Regeneration,Shanghai 200072,China

出　　处：《Frontiers of Medicine》2021年第5期657-678,共22页医学前沿（英文版）

基　　金：This work was supported by grants from the National Natural Science Foundation of China(Nos.82061130222,81822012,81771043,92049201,81770873,and 81802193);the National Science and Technology Major Project of China(No.2016YFC1102705);the Shanghai Academic Leader of Science and Technology Innovation Action Plan(No.20XD1424000);the Shanghai Experimental Animal Research Project of Science and Technology Innovation Action Plan(No.8191101676).

摘　　要：Bone and teeth are hard tissues.Hard tissue diseases have a serious effect on human survival and quality of life.Primary cilia are protrusions on the surfaces of cells.As antennas,they are distributed on the membrane surfaces of almost all mammalian cell types and participate in the development of organs and the maintenance of homeostasis.Mutations in cilium-related genes result in a variety of developmental and even lethal diseases.Patients with multiple ciliary gene mutations present overt changes in the skeletal system,suggesting that primary cilia are involved in hard tissue development and reconstruction.Furthermore,primary cilia act as sensors of external stimuli and regulate bone homeostasis.Specifically,substances are trafficked through primary cilia by intraflagellar transport,which affects key signaling pathways during hard tissue development.In this review,we summarize the roles of primary cilia in long bone development and remodeling from two perspectives:primary cilia signaling and sensory mechanisms.In addition,the cilium-related diseases of hard tissue and the manifestations of mutant cilia in the skeleton and teeth are described.We believe that all the findings will help with the intervention and treatment of related hard tissue genetic diseases.

关 键 词：primary cilia BONE mechanical sensing hard tissue cilium-related bone disease TOOTH 

分 类 号：R68[医药卫生—骨科学]