A role for the BBSome in restricting the entry of non-ciliary proteins into cilia
Abstract
Cilia possess the inherent ability to restrict the entry of non-ciliary components through the ciliary gate. Mutations in genes encoding components of the ciliary gate result in various cilia-related diseases known as ciliopathies. Yet, how non-ciliary residents are prevented from accumulating in cilia remains unclear. Here, we observe ciliary accumulation of non-ciliary proteins in BBSome-deficient mutants (Bardet-Biedl syndrome), a phenotype reminiscent of ciliary gate-defective mutants such as mks-5 and nphp-4 . Time-lapse imaging shows that these non-ciliary proteins move bidirectionally along cilia at IFT velocities, and bimolecular fluorescence complementation (BiFC) reveals that the non-ciliary protein ELMD-1 associates with the IFT component IFT-43 in BBSome-deficient mutants, indicating that they are mis-loaded onto IFT trains. This misloading allows them to bypass the ciliary gate. Fluorescence recovery after photobleaching (FRAP) suggests the gate remains largely functional, supporting a potential two-step model of ciliary composition control: cargo selection/loading and ciliary gating. We propose that defects in cargo selection/loading (and potentially export) alter ciliary composition, allowing unintended protein entry.
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BibTeX
@article{cevik2026role,
title = {{A role for the BBSome in restricting the entry of non-ciliary proteins into cilia}},
author = {Sebiha Cevik and Ferhan Yenisert and Hanife Kantarci and Damla Pucak and Christian Gallrein and Simon Stöckl and Xenia Dünninger and Onur Cakici and Christian Stigloher and Janine Kirstein and Oktay I. Kaplan},
journal = {Communications Biology},
year = {2026},
month = oct,
doi = {10.1038/s42003-026-10986-3},
url = {https://www.nature.com/articles/s42003-026-10986-3},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - A role for the BBSome in restricting the entry of non-ciliary proteins into cilia
AU - Sebiha Cevik
AU - Ferhan Yenisert
AU - Hanife Kantarci
AU - Damla Pucak
AU - Christian Gallrein
AU - Simon Stöckl
AU - Xenia Dünninger
AU - Onur Cakici
AU - Christian Stigloher
AU - Janine Kirstein
AU - Oktay I. Kaplan
PY - 2026
DA - 2026/10/25
JO - Communications Biology
DO - 10.1038/s42003-026-10986-3
UR - https://www.nature.com/articles/s42003-026-10986-3
AB - Cilia possess the inherent ability to restrict the entry of non-ciliary components through the ciliary gate. Mutations in genes encoding components of the ciliary gate result in various cilia-related diseases known as ciliopathies. Yet, how non-ciliary residents are prevented from accumulating in cilia remains unclear. Here, we observe ciliary accumulation of non-ciliary proteins in BBSome-deficient mutants (Bardet-Biedl syndrome), a phenotype reminiscent of ciliary gate-defective mutants such as mks-5 and nphp-4 . Time-lapse imaging shows that these non-ciliary proteins move bidirectionally along cilia at IFT velocities, and bimolecular fluorescence complementation (BiFC) reveals that the non-ciliary protein ELMD-1 associates with the IFT component IFT-43 in BBSome-deficient mutants, indicating that they are mis-loaded onto IFT trains. This misloading allows them to bypass the ciliary gate. Fluorescence recovery after photobleaching (FRAP) suggests the gate remains largely functional, supporting a potential two-step model of ciliary composition control: cargo selection/loading and ciliary gating. We propose that defects in cargo selection/loading (and potentially export) alter ciliary composition, allowing unintended protein entry.
SN - 2399-3642
ER -