Centrosomes and cilia in human disease.
TLDR
There is very little overlap between the genes affected in the different diseases, suggesting that there are tissue-specific requirements for these microtubule-derived structures, focusing on cancer, diseases of brain development and ciliopathies.About:
This article is published in Trends in Genetics.The article was published on 2011-08-01 and is currently open access. It has received 332 citations till now. The article focuses on the topics: Centriole & Centrosome.read more
Citations
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The Cell Biology of Neurogenesis: Toward an Understanding of the Development and Evolution of the Neocortex
TL;DR: The issue of cell polarity is discussed, as well as specific subcellular features of these cells that are relevant for their modes of division and daughter cell fate, which help gain insight into key developmental and evolutionary mechanisms underlying neocortex expansion.
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The centrosome cycle: Centriole biogenesis, duplication and inherent asymmetries
Erich A. Nigg,Tim Stearns +1 more
TL;DR: The spatial aspects of the centrosome duplication cycle, the mechanism of centriole assembly and the possible consequences of the inherent asymmetry of Centrosomes and centrosomes are discussed.
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Subdiffraction imaging of centrosomes reveals higher-order organizational features of pericentriolar material
Steffen Lawo,Steffen Lawo,Monica Hasegan,Gagan D. Gupta,Laurence Pelletier,Laurence Pelletier +5 more
TL;DR: It is found that PCM components occupy separable spatial domains within mitotic PCM that are maintained in the absence of microtubule nucleation complexes and further implicate PCNT and CDK5RAP2 in the organization and assembly of PCM.
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Subdiffraction-resolution fluorescence microscopy reveals a domain of the centrosome critical for pericentriolar material organization
Vito Mennella,Bettina Keszthelyi,Kent L. McDonald,Bryant B. Chhun,F Kan,Gregory C. Rogers,Bo Huang,David A. Agard +7 more
TL;DR: It is demonstrated that the pericentriolar material is organized into two main structural domains: a layer juxtaposed to the centriole wall, and proteins extending farther away from the Centrosome organized in a matrix, using SIM and STORM subdiffraction-resolution microscopies.
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Once and only once: mechanisms of centriole duplication and their deregulation in disease
Erich A. Nigg,Andrew J. Holland +1 more
TL;DR: A better understanding of the molecular mechanisms governing centriole biogenesis is understood, opening up new possibilities for targeting these pathways in the context of human disease.
References
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The primary cilium: a signalling centre during vertebrate development
TL;DR: The connections between cilia and developmental signalling have begun to clarify the basis of human diseases associated with ciliary dysfunction, and the cilium represents a nexus for signalling pathways during development.
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Randomization of Left–Right Asymmetry due to Loss of Nodal Cilia Generating Leftward Flow of Extraembryonic Fluid in Mice Lacking KIF3B Motor Protein
Shigenori Nonaka,Yosuke Tanaka,Yasushi Okada,Sen Takeda,Akihiro Harada,Yoshimitsu Kanai,Mizuho A. Kido,Nobutaka Hirokawa +7 more
TL;DR: The data suggest that KIF3B is essential for the left-right determination through intraciliary transportation of materials for ciliogenesis of motile primary cilia that could produce a gradient of putative morphogen along the left–right axis in the node.
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A Mechanism Linking Extra Centrosomes to Chromosomal Instability
TL;DR: It is demonstrated that cells with multiple centrosomes rarely undergo multipolar cell divisions, and the progeny of these divisions are typically inviable, and it is proposed that this mechanism may be a common underlying cause of CIN in human cancer.
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Chlamydomonas IFT88 and Its Mouse Homologue, Polycystic Kidney Disease Gene Tg737, Are Required for Assembly of Cilia and Flagella
Gregory J. Pazour,Bethany L. Dickert,Yvonne Vucica,E. Scott Seeley,Joel L. Rosenbaum,George B. Witman,Douglas G. Cole +6 more
TL;DR: The primary cilia in the kidney of Tg737 mutant mice are shorter than normal, indicating that IFT is important forPrimary cilia assembly in mammals and that defects in their assembly can lead to polycystic kidney disease.
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The Polycystic Kidney Disease Proteins, Polycystin-1, Polycystin-2, Polaris, and Cystin, Are Co-Localized in Renal Cilia
TL;DR: Co-localization in cilia of polycystin-1 and polycyStin-2 is demonstrated, which is the principal proteins involved in autosomal dominant polycystic kidney disease, with polaris and cystin, which are proteins that are disrupted in the Tg737(orpk)and cpk mouse models of autosomal recessive polycysts disease, respectively.