Arl13b and the exocyst interact synergistically in ciliogenesis [PDF]
Arl13b belongs to the ADP-ribosylation factor family within the Ras superfamily of regulatory GTPases. Mutations in Arl13b cause Joubert syndrome, which is characterized by congenital cerebellar ataxia, hypotonia, oculomotor apraxia, and mental retardation.
Cecília Seixas+13 more
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ARL13B promotes angiogenesis and glioma growth by activating VEGFA-VEGFR2 signaling
Abstract Background Tumor angiogenesis is essential for solid tumor progression, invasion and metastasis. The aim of this study was to identify potential signaling pathways involved in tumor angiogenesis.
Limin Chen+12 more
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A G-protein activation cascade from Arl13B to Arl3 and implications for ciliary targeting of lipidated proteins [PDF]
Small G-proteins of the ADP-ribosylation-factor-like (Arl) subfamily have been shown to be crucial to ciliogenesis and cilia maintenance. Active Arl3 is involved in targeting and releasing lipidated cargo proteins from their carriers PDE6δ and UNC119a/b ...
Katja Gotthardt+5 more
doaj +3 more sources
Cilia localization is essential for in vivo functions of the Joubert syndrome protein Arl13b/Scorpion [PDF]
arl13b was initially cloned as the novel cystic kidney gene scorpion (sco) in zebrafish and was shown to be required for cilia formation in the kidney duct. In mouse, a null mutant of Arl13b shows abnormal ultrastructure of the cilium and defective sonic hedgehog (Shh) signaling.
Neil A. Duldulao+2 more
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The Joubert Syndrome Gene arl13b is Critical for Early Cerebellar Development in Zebrafish [PDF]
AbstractJoubert syndrome is characterized by unique malformation of the cerebellar vermis. More than thirty Joubert syndrome genes have been identified, includingARL13B. However, its role in cerebellar development remains unexplored. We found that knockdown or knockout ofarl13bimpaired balance and locomotion in zebrafish larvae.
Hong A Xu, Víctor Vinuesa
exaly +4 more sources
Methods to Assess Neuronal Primary Cilia Electrochemical Signaling [PDF]
Hippocampal neuron primary cilia are illuminated with a fluorescent biosensor. ABSTRACT Primary cilia are polymodal sensory organelles which project from the apical side of polarized cells. They are found in all brain hemispheres but are most pronounced in neurons, which comprise the granular layers of the hippocampus and cerebellum.
Paul G. DeCaen, Louise F. Kimura
wiley +2 more sources
Ciliary ARL13B inhibits developmental kidney cystogenesis in mouse
AbstractARL13B is a small GTPase enriched in cilia. Deletion ofArl13bin mouse kidney results in renal cysts and an associated absence of primary cilia. Similarly, ablation of cilia leads to kidney cysts. To investigate whether ARL13B functions from within cilia to direct kidney development, we examined kidneys of mice expressing an engineered cilia ...
Robert E. Van Sciver+4 more
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Role of ciliary proteins ADP Ribosylation Factor Like GTPase 13B (ARL13B) and Bardet-Biedl Syndrome-8 (BBS8) in photoreceptor outer segment morphogenesis, maintenance, and viability [PDF]
Photoreceptor neurons are modified primary cilia with an extended ciliary compartment known as the outer segment (OS). The mechanisms behind the elaboration of photoreceptor cilia, OS morphogenesis, and maintenance remain poorly understood. In this work,
Dilan, Tanya L
core +3 more sources
ARL13B, PDE6D, and CEP164 form a functional network for INPP5E ciliary targeting [PDF]
Mutations affecting ciliary components cause a series of related genetic disorders in humans, including nephronophthisis (NPHP), Joubert syndrome (JBTS), Meckel-Gruber syndrome (MKS), and Bardet-Biedl syndrome (BBS), which are collectively termed “ciliopathies.” Recent protein–protein interaction studies combined with genetic analyses revealed that ...
Melissa C. Humbert+6 more
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The small GTPase ARL13B is mutated in the human ciliopathy Joubert syndrome. Here the authors show that ARL13B and the RAB11 effector FIP5 promote import of tubulin glutamylase into cilia and as such axoneme polyglutamylation, which is demonstrated to be
Jian Zhao, Kun Ling, Jinghua Hu
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