RRC ID 6946
著者 Cevik S, Hori Y, Kaplan OI, Kida K, Toivenon T, Foley-Fisher C, Cottell D, Katada T, Kontani K, Blacque OE.
タイトル Joubert syndrome Arl13b functions at ciliary membranes and stabilizes protein transport in Caenorhabditis elegans.
ジャーナル J Cell Biol
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.
巻・号 188(6)
ページ 953-69
公開日 2010-3-22
DOI 10.1083/jcb.200908133
PII jcb.200908133
PMID 20231383
PMC PMC2845074
MeSH ADP-Ribosylation Factors / metabolism* Animals Caenorhabditis elegans / metabolism* Cell Membrane / metabolism* Cells, Cultured Cilia / genetics Cilia / metabolism Dogs Humans Protein Transport
IF 8.811
引用数 110
WOS 分野 CELL BIOLOGY
リソース情報
線虫 tm2322