Chinese Journal of Tissue Engineering Research ›› 2016, Vol. 20 ›› Issue (24): 3642-3648.doi: 10.3969/j.issn.2095-4344.2016.24.022

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Ciliopathies: cellular mechanism and translational application

Na Yu-yan1, Liu Wan-lin2   

  1. 1Inner Mongolia Medical University, Hohhot 010059, Inner Mongolia Autonomous Region, China; 2Department of Orthopedics, the Second Affiliated Hospital of Inner Mongolia Medical University, Hohhot 010030, Inner Mongolia Autonomous Region, China
  • Online:2016-06-10 Published:2016-06-10
  • Contact: Liu Wan-lin, Professor, Chief physician, Doctoral supervisor, Department of Orthopedics, the Second Affiliated Hospital of Inner Mongolia Medical University, Hohhot 010030, Inner Mongolia Autonomous Region, China
  • About author:Na Yu-yan, Studying for master’s degree, Inner Mongolia Medical University, Hohhot 010059, Inner Mongolia Autonomous Region, China
  • Supported by:

    the National Natural Science Foundation of China, No. 81560349

Abstract:

BACKGROUND: Cilium is an antenna-like organelle that plays a vital role in all organs generation and maintenance. Ciliopathies refer to multisystem diseases caused by disorders of the cilia structure and function.
OBJECTIVE: To introduce current understanding of the structure and function of the cilium, with a focus on the study progress of ciliopathies.
METHODS: PubMed database was searched by computer using the keywords of “primary cilium, motion cilium, ciliopathy”. Totally 371 relevant articles published from 1959 to 2015 were retrieved. After excluding repetitive and irrelevant articles, we enrolled 32 English literatures regarding cilia structure, function and ciliopathies in accordance with the inclusion criteria.
RESULTS AND CONCLUSION: Ciliopathies almost involve every system of the human body, such as the brain, eyes, liver, kidney, bone and reproductive system. Most ciliopathies are related to human developmental or degenerative diseases, which characterized by wide-ranging clinical and genetic heterogeneity, such as primary ciliary dyskinesia, ploycystic kidney disease, infertility, blindness and tumor. In view of the medical importance of the cilium, scholars have devoted to studying the underlying cellular mechanisms. These in turn have revealed that ciliopathies are not only caused by defective assembly of the cilium, but also by impaired ciliary signaling.

中国组织工程研究杂志出版内容重点:组织构建;骨细胞;软骨细胞;细胞培养;成纤维细胞;血管内皮细胞;骨质疏松组织工程

Key words: Cilia, Ciliary Motility Disorders, Tissue Engineering

CLC Number: