---
pmid: '25860617'
title: Characterization of tetratricopeptide repeat-containing proteins critical for
  cilia formation and function.
authors:
- Xu Y
- Cao J
- Huang S
- Feng D
- Zhang W
- Zhu X
- Yan X
journal: PLoS One
year: '2015'
full_text_available: false
pmcid: PMC4393279
doi: 10.1371/journal.pone.0124378
pubmed_publication_types:
- Journal Article
- Research Support, Non-U.S. Gov't
publication_type: PRIMARY_RESEARCH
---

# Characterization of tetratricopeptide repeat-containing proteins critical for cilia formation and function.
**Authors:** Xu Y, Cao J, Huang S, Feng D, Zhang W, Zhu X, Yan X
**Journal:** PLoS One (2015)
**DOI:** [10.1371/journal.pone.0124378](https://doi.org/10.1371/journal.pone.0124378)
**PMC:** [PMC4393279](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4393279/)

## Abstract

1. PLoS One. 2015 Apr 10;10(4):e0124378. doi: 10.1371/journal.pone.0124378.
eCollection 2015.

Characterization of tetratricopeptide repeat-containing proteins critical for
cilia formation and function.

Xu Y(1), Cao J(1), Huang S(1), Feng D(2), Zhang W(1), Zhu X(3), Yan X(1).

Author information:
(1)State Key Laboratory of Cell Biology, Institute of Biochemistry and Cell
Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of
Sciences, 320 Yueyang Road, Shanghai, China.
(2)School of Life Science and Technology, ShanghaiTech University, 100 Haike
Road, Shanghai, China.
(3)State Key Laboratory of Cell Biology, Institute of Biochemistry and Cell
Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of
Sciences, 320 Yueyang Road, Shanghai, China; School of Life Science and
Technology, ShanghaiTech University, 100 Haike Road, Shanghai, China.

Cilia formation and function require a special set of trafficking machinery
termed intraflagellar transport (IFT), consisting mainly of protein complexes
IFT-A, IFT-B, BBSome, and microtubule-dependent molecular motors.
Tetratricopeptide repeat-containing (TTC) proteins are widely involved in
protein complex formation. Nine of them are known to serve as components of the
IFT or BBSome complexes. How many TTC proteins are cilia-related and how they
function, however, remain unclear. Here we show that twenty TTC genes were
upregulated by at least 2-fold during the differentiation of cultured mouse
tracheal epithelial cells (MTECs) into multiciliated cells. Our systematic
screen in zebrafish identified four novel TTC genes, ttc4, -9c, -36, and -39c,
that are critical for cilia formation and motility. Accordingly, their zebrafish
morphants displayed typical ciliopathy-related phenotypes, including curved
body, abnormal otolith, hydrocephalus, and defective left-right patterning. The
morphants of ttc4 and ttc25, a known cilia-related gene, additionally showed
pronephric cyst formation. Immunoprecipitation indicated associations of TTC4,
-9c, -25, -36, and -39c with components or entire complexes of IFT-A, IFT-B, or
BBSome, implying their participations in IFT or IFT-related activities. Our
results provide a global view for the relationship between TTC proteins and
cilia.

DOI: 10.1371/journal.pone.0124378
PMCID: PMC4393279
PMID: 25860617 [Indexed for MEDLINE]

Conflict of interest statement: Competing Interests: The authors have declared
that no competing interests exist.