RRC ID 53317
Author Wei Q, Zhang Y, Schouteden C, Zhang Y, Zhang Q, Dong J, Wonesch V, Ling K, Dammermann A, Hu J.
Title The hydrolethalus syndrome protein HYLS-1 regulates formation of the ciliary gate.
Journal Nat Commun
Abstract Transition fibres (TFs), together with the transition zone (TZ), are basal ciliary structures thought to be crucial for cilium biogenesis and function by acting as a ciliary gate to regulate selective protein entry and exit. Here we demonstrate that the centriolar and basal body protein HYLS-1, the C. elegans orthologue of hydrolethalus syndrome protein 1, is required for TF formation, TZ organization and ciliary gating. Loss of HYLS-1 compromises the docking and entry of intraflagellar transport (IFT) particles, ciliary gating for both membrane and soluble proteins, and axoneme assembly. Additional depletion of the TF component DYF-19 in hyls-1 mutants further exacerbates TZ anomalies and completely abrogates ciliogenesis. Our data support an important role for HYLS-1 and TFs in establishment of the ciliary gate and underline the importance of selective protein entry for cilia assembly.
Volume 7
Pages 12437
Published 2016-8-18
DOI 10.1038/ncomms12437
PII ncomms12437
PMID 27534274
PMC PMC4992140
MeSH Animals Caenorhabditis elegans Caenorhabditis elegans Proteins / metabolism* Cilia / metabolism* Cilia / ultrastructure Flagella / metabolism Gene Deletion Hand Deformities, Congenital / metabolism* Heart Defects, Congenital / metabolism* Hydrocephalus / metabolism* Models, Biological Organogenesis
IF 11.878
Times Cited 10
C.elegans tm3067 tm2705 tm2547 tm3100