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

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.

SUBMITTER: Wei Q

PROVIDER: S-EPMC4992140 | biostudies-literature | 2016 Aug

REPOSITORIES: biostudies-literature

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

Wei Qing Q Zhang Yingyi Y Schouteden Clementine C Zhang Yuxia Y Zhang Qing Q Dong Jinhong J Wonesch Veronika V Ling Kun K Dammermann Alexander A Hu Jinghua J

Nature communications 20160818

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 transpor ...[more]

PMID: 27534274

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The hydrolethalus syndrome protein HYLS-1 links core centriole structure to cilia formation.

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| S-EPMC6174286 | biostudies-literature

Dysfunction of the ciliary ARMC9/TOGARAM1 protein module causes Joubert syndrome.

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| S-EPMC7410078 | biostudies-literature

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