Regulation of ciliary polarity by the APC/C

Ganner, Athina; Lienkamp, Soeren S.; Schäfer, Tobias; Romaker, Daniel; Węgierski, Tomasz; Park, Tae Joo; Spreitzer, Stefan; Simons, Matias; Gloy, Joachim; Kim, Emily; Wallingford, John B.; Walz, Gerd

doi:10.1073/pnas.0909465106

Cited by 49 publications

(46 citation statements)

References 37 publications

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“…Wnt/␤-catenin signaling activation induces dorsal axis duplication in Xenopus embryos (5,9,12). Consistent with previous reports (12), injection of wild-type or AY mutant Dvl2 mRNA could induce a second axis. However, co-injection of NEDD4L mRNA repressed axis duplication induced by wild-type Dvl2, but not by the AY mutant Dvl2 (Fig.…”

Section: Nedd4lsupporting

confidence: 79%

HECT Domain-containing E3 Ubiquitin Ligase NEDD4L Negatively Regulates Wnt Signaling by Targeting Dishevelled for Proteasomal Degradation

Ding

Zhang

et al. 2013

Journal of Biological Chemistry

104

100

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Background: Dishevelled is a critical component of Wnt signaling; however, its stability control is not fully understood. Results: NEDD4L regulates Wnt signaling through Dishevelled2 degradation, and Wnt5a-induced NEDD4L phosphorylation by JNK1 is required for this process. Conclusion: NEDD4L modulates Wnt signaling through a negative feedback mechanism. Significance: Our findings shed light on the understanding of Dishevelled2 stability control and NEDD4L-associated diseases.

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Section: Nedd4lsupporting

confidence: 79%

HECT Domain-containing E3 Ubiquitin Ligase NEDD4L Negatively Regulates Wnt Signaling by Targeting Dishevelled for Proteasomal Degradation

Ding

Zhang

et al. 2013

Journal of Biological Chemistry

104

100

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“…The gene encodes one of the several exocyst proteins that are recruited to the basal body of budding cilia along with the members of disheveled family of proteins in order to dock the basal body to the membrane and allow the nascent cilia to form. 42,43 Knockdown of another exocyst gene SEC10 was found to impair ciliogenesis, raising the possibility that defective SEC8 may lead a similar cellular phenotype. Furthermore, another exocyst gene EXOC8 was recently found mutated in a patient with Joubert syndrome.…”

Section: Head and Neckmentioning

confidence: 99%

Genomic analysis of Meckel–Gruber syndrome in Arabs reveals marked genetic heterogeneity and novel candidate genes

et al. 2012

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Meckel-Gruber syndrome (MKS, OMIM #249000) is a multiple congenital malformation syndrome that represents the severe end of the ciliopathy phenotypic spectrum. Despite the relatively common occurrence of this syndrome among Arabs, little is known about its genetic architecture in this population. This is a series of 18 Arab families with MKS, who were evaluated clinically and studied using autozygome-guided mutation analysis and exome sequencing. We show that autozygome-guided candidate gene analysis identified the underlying mutation in the majority (n ¼ 12, 71%). Exome sequencing revealed a likely pathogenic mutation in three novel candidate MKS disease genes. These include C5orf42, Ellis-van-Creveld disease gene EVC2 and SEC8 (also known as EXOC4), which encodes an exocyst protein with an established role in ciliogenesis. This is the largest and most comprehensive genomic study on MKS in Arabs and the results, in addition to revealing genetic and allelic heterogeneity, suggest that previously reported disease genes and the novel candidates uncovered by this study account for the overwhelming majority of MKS patients in our population.

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“…Expression of the Dvl2 dominant-negative construct Xdd1 in the Xenopus skin disrupts BB orientation without affecting docking, supporting that these two aspects of Dvl2 function can be uncoupled (Park et al 2008). Accumulation of Dvl2 at the base of cilia following inactivation of the anaphase-promoting complex, which targets Dvl2 for degradation, results in BB disorientation in the Xenopus skin, suggesting that BB polarization requires a tight control of Dvl2 levels at the BBs (Ganner et al 2009). Besides Dvl2, diversin is present at the BBs in mouse airway and Xenopus skin MCCs (Yasunaga et al 2011;Vladar et al 2012), and Pk2 associates to BBs in mature Xenopus skin MCCs (Butler and Wallingford 2015).…”

Section: Polarization By the Pcp Pathwaymentioning

confidence: 99%

Multiciliated Cells in Animals

Meunier¹,

Azimzadeh²

2016

Cold Spring Harb Perspect Biol

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Many animal cells assemble single cilia involved in motile and/or sensory functions. In contrast, multiciliated cells (MCCs) assemble up to 300 motile cilia that beat in a coordinate fashion to generate a directional fluid flow. In the human airways, the brain, and the oviduct, MCCs allow mucus clearance, cerebrospinal fluid circulation, and egg transportation, respectively. Impairment of MCC function leads to chronic respiratory infections and increased risks of hydrocephalus and female infertility. MCC differentiation during development or repair involves the activation of a regulatory cascade triggered by the inhibition of Notch activity in MCC progenitors. The downstream events include the simultaneous assembly of a large number of basal bodies (BBs)-from which cilia are nucleated-in the cytoplasm of the differentiating MCCs, their migration and docking at the plasma membrane associated to an important remodeling of the actin cytoskeleton, and the assembly and polarization of motile cilia. The direction of ciliary beating is coordinated both within cells and at the tissue level by a combination of planar polarity cues affecting BB position and hydrodynamic forces that are both generated and sensed by the cilia. Herein, we review the mechanisms controlling the specification and differentiation of MCCs and BB assembly and organization at the apical surface, as well as ciliary assembly and coordination in MCCs.

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Regulation of ciliary polarity by the APC/C

Cited by 49 publications

References 37 publications

HECT Domain-containing E3 Ubiquitin Ligase NEDD4L Negatively Regulates Wnt Signaling by Targeting Dishevelled for Proteasomal Degradation

HECT Domain-containing E3 Ubiquitin Ligase NEDD4L Negatively Regulates Wnt Signaling by Targeting Dishevelled for Proteasomal Degradation

Genomic analysis of Meckel–Gruber syndrome in Arabs reveals marked genetic heterogeneity and novel candidate genes

Multiciliated Cells in Animals

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