The primary cilium as a multiple cellular signaling scaffold in development and disease

• Published in: BMB reports Vol. 45; no. 8; pp. 427 - 432
• Main Author: Ko, H.W., College of Pharmacy, Dongguk University, Goyang, Republic of Korea
• Format: Journal Article
• Language: English
• Published: Korea (South) Korean Society for Biochemistry and Molecular Biology 01-08-2012; 생화학분자생물학회
• Subjects: Animals; Cell Cycle Proteins - metabolism; Centrioles - metabolism; Cilia - chemistry; Cilia - metabolism; Ciliary length control; Ciliogenesis; Ciliopathy; ERIZO; Hedgehog; HEDGEHOGS; HERISSON; Kidney Diseases, Cystic - metabolism; Kidney Diseases, Cystic - pathology; Microtubules - metabolism; Primary cilia; Signal Transduction; Wnt; 화학
• ISSN: 1976-6696; 1976-670X
• DOI: 10.5483/BMBRep.2012.45.8.167

Primary cilia, single hair-like appendage on the surface of the most mammalian cells, were once considered to be vestigial cellular organelles for a past century because of their tiny structure and unknown function. Although they lack ancestral motility function of cilia or flagella, they share common ground with multiciliated motile cilia and flagella on internal structure such as microtubule based nine outer doublets nucleated from the base of mother centrioles called basal body. Making cilia, ciliogenesis, in cells depends on the cell cycle stage due to reuse of centrioles for cell division forming mitotic spindle pole (M phase) and assembling cilia from basal body (starting G1 phase and maintaining most of interphase). Ciliary assembly required two conflicting processes such as assembly and disassembly and balance between these two processes determines the length of cilia. Both process required highly conserved transport system to supply needed substance to grow tip of cilia and bring ciliary turnover product back to the base of cilia using motor protein, kinesin and dynein, and transport protein complex, IFT particles. Disruption of ciliary structure or function causes multiple human disorder called ciliopathies affecting disease of diverse ciliated tissues ranging from eye, kidney, respiratory tract and brain. Recent explosion of research on the primary cilia and their involvement on animal development and disease attracts scientific interest on how extensively the function of cilia related to specific cell physiology and signaling pathway. In this review, I introduce general features of primary cilia and recent progress in understanding of the ciliary length control and signaling pathways transduced through primary cilia in vertebrates.