2. Academic Validation
  3. Cilia locally synthesize proteins to sustain their ultrastructure and functions

Cilia locally synthesize proteins to sustain their ultrastructure and functions

  • Nat Commun. 2021 Nov 30;12(1):6971. doi: 10.1038/s41467-021-27298-1.
Kai Hao 1 2,  Yawen Chen 1 2,  Xiumin Yan 3,  Xueliang Zhu 4 5 6
Affiliations

Affiliations

  • 1 State Key Laboratory of Cell Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, 320 Yueyang Road, 200031, Shanghai, China.
  • 2 University of Chinese Academy of Sciences, 100049, Beijing, China.
  • 3 Ministry of Education-Shanghai Key Laboratory of Children's Environmental Health, Institute of Early Life Health, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, 200092, Shanghai, China. yanxiumin@xinhuamed.com.cn.
  • 4 State Key Laboratory of Cell Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, 320 Yueyang Road, 200031, Shanghai, China. xlzhu@sibcb.ac.cn.
  • 5 University of Chinese Academy of Sciences, 100049, Beijing, China. xlzhu@sibcb.ac.cn.
  • 6 School of Life Science, Hangzhou Institute for Advanced Study, University of Chinese Academy of Sciences, 310024, Hangzhou, China. xlzhu@sibcb.ac.cn.
PMID: 34848703 DOI: 10.1038/s41467-021-27298-1
Abstract

Cilia are microtubule-based hair-like organelles propelling locomotion and extracellular liquid flow or sensing environmental stimuli. As cilia are diffusion barrier-gated subcellular compartments, their protein components are thought to come from the cell body through intraflagellar transport or diffusion. Here we show that cilia locally synthesize proteins to maintain their structure and functions. Multicilia of mouse ependymal cells are abundant in ribosomal proteins, translation initiation factors, and RNA, including 18 S rRNA and tubulin mRNA. The cilia actively generate nascent Peptides, including those of tubulin. mRNA-binding protein Fmrp localizes in ciliary central lumen and appears to function in mRNA delivery into the cilia. Its depletion by RNAi impairs ciliary local translation and induces multicilia degeneration. Expression of exogenous Fmrp, but not an isoform tethered to mitochondria, rescues the degeneration defects. Therefore, local translation defects in cilia might contribute to the pathology of ciliopathies and Other Diseases such as Fragile X syndrome.

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