Ccdc11 is a novel centriolar satellite protein essential for ciliogenesis and establishment of left–right asymmetry

@article{Silva2016Ccdc11IA,
  title={Ccdc11 is a novel centriolar satellite protein essential for ciliogenesis and establishment of left–right asymmetry},
  author={Erica Silva and Ewelina Betleja and E John and Philip C Spear and James J. Moresco and Siwei Zhang and John R. Yates and Brian J Mitchell and Moe R Mahjoub},
  journal={Molecular Biology of the Cell},
  year={2016},
  volume={27},
  pages={48 - 63}
}
Mutations in CCDC11 cause aberrant placement of internal organs and congenital heart disease in humans. Ccdc11 is a novel component of centriolar satellites and plays a critical role in motile and sensory ciliogenesis. The results implicate centriolar satellites in the pathology of left–right patterning and heart disease. 
The Cilium- and Centrosome-Associated Protein CCDC11 Is Required for Cytokinesis via Midbody Recruitment of the ESCRT- III Membrane Scission Complex Subunit CHMP2A
Introduction The coiled-coil domain-containing 11 (CCDC11) protein is a 62-kDa polypeptide that is associated with both ciliary and centriolar satellite structures (1-3). Cilia are microtubule-basedExpand
The centriolar satellite protein CCDC66 interacts with CEP290 and functions in cilium formation and trafficking
TLDR
The retinal degeneration gene product CCDC66 is a microtubule-associated protein that dynamically localizes to the centrosome, centriolar satellites and the primary cilium throughout the cell cycle, and functions in cilum formation and recruitment of BBS4 to thecilium. Expand
Roles of the cilium-associated gene CCDC11 in left-right patterning and in laterality disorders in humans.
TLDR
The roles of CCDC11 and the implications of the identified mutation on left-right axial patterning in patient-derived cells and in the frog embryo as a model organism are characterized. Expand
The ciliopathy gene product Cep290 is required for primary cilium formation and microtubule network organization.
TLDR
The function and regulation of Cep290, which is the most frequently mutated gene in ciliopathies and importantly its functions remain poorly understood, are elucidated using the CRISPR/Cas9 genome editing approach and phenotypic insight into the disease mechanisms of CEP290 ciliopathy mutations is provided. Expand
Centriolar satellites are required for efficient ciliogenesis and ciliary content regulation
TLDR
This work generated a kidney epithelial cell line lacking satellites by CRISPR/Cas9‐mediated PCM1 deletion and investigated the cellular and molecular consequences of satellite loss, finding that loss of satellites affects transcription scarcely, but significantly alters the proteome. Expand
A novel Cep120-dependent mechanism inhibits centriole maturation in quiescent cells
TLDR
The results indicate that Cep120 helps to maintain centrosome homeostasis by inhibiting untimely maturation of the daughter centriole, and defines a potentially new molecular defect underlying the pathogenesis of ciliopathies such as Jeune Asphyxiating Thoracic Dystrophy and Joubert syndrome. Expand
Regulation of centriolar satellite integrity and its physiology
TLDR
The most recent findings highlight dynamic structural remodelling in response to internal and external cues and unexpected positive feedback control that is exerted from the centrosome for centriolar satellite integrity. Expand
Centriolar satellite biogenesis and function in vertebrate cells
TLDR
The evolution from thinking of satellites as mere facilitators of protein trafficking to the centrosome to thinking of them being key regulators of protein localization and cellular proteostasis for a diverse set of pathways is discussed, making them of broader interest to fields beyond those focused on centrosomes and ciliogenesis. Expand
Acute inhibition of centriolar satellite function and positioning reveals their functions at the primary cilium
TLDR
A chemical-based trafficking assay was developed to rapidly and efficiently redistribute satellites to either the cell periphery or center, and fuse them into stable clusters in a temporally-controlled way, and provided a new tool for probing temporal satellite functions in different contexts. Expand
The Heterotaxy Gene CCDC11 is Essential for Cytokinesis and Cell-Cell Adhesion via RhoA Regulation
TLDR
The results suggest that CCDC11 connects cytokinesis and LR patterning via RhoA regulation, providing a potential mechanism for heterotaxy disease pathogenesis. Expand
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