Ccdc57 is required for straightening the body axis by regulating ciliary motility in the brain ventricle of zebrafish.
J Genet Genomics
; 50(4): 253-263, 2023 04.
Article
in En
| MEDLINE
| ID: mdl-36669737
ABSTRACT
Recently, cilia defects have been proposed to contribute to scoliosis. Here, we demonstrate that coiled-coil domain-containing 57 (Ccdc57) plays an essential role in straightening the body axis of zebrafish by regulating ciliary beating in the brain ventricle (BV). Zygotic ccdc57 (Zccdc57) mutant zebrafish developes scoliosis without significant changes in their bone density and calcification, and the maternal-zygotic ccdc57 (MZccdc57) mutant embryos display curved bodies since the long-pec stage. The expression of ccdc57 is enriched in ciliated tissues and immunofluorescence analysis reveals colocalization of Ccdc57-HA with acetylated α-tubulin, implicating it in having a role in ciliary function. Further examination reveals that it is the coordinated cilia beating of multiple cilia bundles (MCB) in the MZccdc57 mutant embryos that is affected at 48 hours post fertilization, when the compromised cerebrospinal fluid flow and curved body axis have already occurred. Either ccdc57 mRNA injection or epinephrine treatment reverses the spinal curvature in MZccdc57 mutant larvae from ventrally curly to straight or even dorsally curly and significantly upregulates urotensin signaling. This study reveals the role of ccdc57 in maintaining coordinated cilia beating of MCB in the BV.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Scoliosis
/
Zebrafish
Limits:
Animals
Language:
En
Journal:
J Genet Genomics
Year:
2023
Document type:
Article
Affiliation country:
China