Helmholtz Gemeinschaft


Joubert syndrome Arl13b functions at ciliary membranes and stabilizes protein transport in Caenorhabditis elegans

[thumbnail of Original Article incl. supplement]
PDF (Original Article incl. supplement) - Requires a PDF viewer such as GSview, Xpdf or Adobe Acrobat Reader
[thumbnail of Supplemental Material] Other (Supplemental Material)

Item Type:Article
Title:Joubert syndrome Arl13b functions at ciliary membranes and stabilizes protein transport in Caenorhabditis elegans
Creators Name:Cevik, S., Hori, Y., Kaplan, O.I., Kida, K., Toivenon, T., Foley-Fisher, C., Cottell, D., Katada, T., Kontani, K. and Blacque, O.E.
Abstract:The small ciliary G protein Arl13b is required for cilium biogenesis and sonic hedgehog signaling and is mutated in patients with Joubert syndrome (JS). In this study, using Caenorhabditis elegans and mammalian cell culture systems, we investigated the poorly understood ciliary and molecular basis of Arl13b function. First, we show that Arl13b/ARL-13 localization is frequently restricted to a proximal ciliary compartment, where it associates with ciliary membranes via palmitoylation modification motifs. Next, we find that loss-of-function C. elegans arl-13 mutants possess defects in cilium morphology and ultrastructure, as well as defects in ciliary protein localization and transport; ciliary transmembrane proteins abnormally accumulate, PKD-2 ciliary abundance is elevated, and anterograde intraflagellar transport (IFT) is destabilized. Finally, we show that arl-13 interacts genetically with other ciliogenic and ciliary transport-associated genes in maintaining cilium structure/morphology and anterograde IFT stability. Together, these data implicate a role for JS-associated Arl13b at ciliary membranes, where it regulates ciliary transmembrane protein localizations and anterograde IFT assembly stability.
Keywords:Cell Membrane, Cilia, Cultured Cells, Protein Transport, Animals, Caenorhabditis elegans, Dogs
Source:Journal of Cell Biology
Publisher:Rockefeller University Press
Page Range:953-969
Date:22 March 2010
Official Publication:https://doi.org/10.1083/jcb.200908133
PubMed:View item in PubMed

Repository Staff Only: item control page


Downloads per month over past year

Open Access
MDC Library