The Hydrolethalus Syndrome Protein HYLS-1 Regulates Formation of the Ciliary Gate

Overview

Journal Nat Commun

Specialty Biology

Date 2016 Aug 19

PMID 27534274

Citations 21

Authors

Qing Wei

Yingyi Zhang

Clementine Schouteden

Yuxia Zhang

Qing Zhang

Jinhong Dong

Veronika Wonesch

Kun Ling

Alexander Dammermann

Jinghua Hu

Affiliations

Soon will be listed here.

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.

Citing Articles

Centriole structural integrity defects are a crucial feature of hydrolethalus syndrome.

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The ARPKD Protein DZIP1L Regulates Ciliary Protein Entry by Modulating the Architecture and Function of Ciliary Transition Fibers.

Chen H, Wu Z, Yan Z, Chen C, Zhang Y, Wang Q Adv Sci (Weinh). 2024; 11(24):e2308820.

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