Makale detayı · 2010
title Joubert syndrome Arl13b functions at ciliary membranes and stabilizes protein transport in i Caenorhabditis elegans /i /title
Dergi
The Journal of Cell BiologyISSN 0021-9525
ISSN kaydı başka bir dergiye işaret ediyor; ad YÖKSİS kaydından.
- Yıl
- 2010
- Tür
- article
Veri kaynağı ayrımı
- YÖKSİS YÖKSİS makale kaydı
- YÖKSİS dergi adı The Journal of Cell Biology
- Katalog eşleşmesi (ISSN) Journal of Cell Biology
- OpenAlex OpenAlex zenginleştirmesi (özet, atıf, konular)
Özet
OpenAlex · İngilizce
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.
Konular
Atıflar
OpenAlex cited_by_count. WoS veya Scopus atıf sayısı değildir; o kaynaklar için ayrı kolon yoktur.
196 atıf
OpenAlex cited_by_count (önbellek / veritabanı)
Yerel katalogda bu makaleye atıf yapan 16 yayın (OpenAlex referans eşleşmesi; tam dünya listesi değildir).
- Active Transport and Diffusion Barriers Restrict Joubert Syndrome-Associated ARL13B/ARL-13 to an Inv-like Ciliary Membrane Subdomain 2013
- Active Transport and Diffusion Barriers Restrict Joubert Syndrome Associated ARL13B ARL 13 to an Inv like Ciliary Membrane Subdomain 2013
- Centriolar satellites are required for efficient ciliogenesis and ciliary content regulation. 2019
- Centriolar satellites are required for efficient ciliogenesis and ciliary content regulation 2019
- Endocytosis Genes Facilitate Protein and Membrane Transport in C. elegans Sensory Cilia 2012
- Endocytosis Genes Facilitate Protein and Membrane Transport in C elegans Sensory Cilia 2012
- The AP-1 clathrin adaptor facilitates cilium formation and functions with RAB-8 in C. elegans ciliary membrane transport 2010
- The AP 1 clathrin adaptor facilitates cilium formation and functions with RAB 8 in C elegans ciliary membrane transport 2010
- ARL13B regulates juxtaposed cilia-cilia elongation in BBSome dependent manner in Caenorhabditis elegans 2025
- ARL13B regulates juxtaposed cilia-cilia elongation in BBSome dependent manner in Caenorhabditis elegans 2025