The hydrolethalus syndrome protein HYLS-1 regulates formation of the ciliary gate

Nat Commun. 2016 Aug 18:7:12437. doi: 10.1038/ncomms12437.

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • 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

Substances

  • Caenorhabditis elegans Proteins
  • HYLS-1 protein, C elegans

Supplementary concepts

  • Hydrolethalus syndrome