A ciliopathy complex builds distal appendages to initiate ciliogenesis
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A ciliopathy complex builds distal appendages to initiate ciliogenesis

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A ciliopathy complex builds distal appendages to initiate ciliogenesis

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dc.contributor.author Kumar, Dhivya
dc.contributor.author Rains, Addison
dc.contributor.author Herranz Pérez, Vicente
dc.contributor.author Qualong, Lu
dc.contributor.author Xiaoyu, Shi
dc.contributor.author Swaney, Danielle L.
dc.contributor.author Stevenson, Erika
dc.contributor.author Krogan, Nevan J.
dc.contributor.author Huang, Bo
dc.contributor.author Westlake, Christopher
dc.contributor.author García Verdugo, José Manuel
dc.contributor.author Yoder, Bradley K.
dc.contributor.author Reiter, Jeremy F.
dc.date.accessioned 2022-04-07T09:45:44Z
dc.date.available 2022-04-07T09:45:44Z
dc.date.issued 2021
dc.identifier.uri https://hdl.handle.net/10550/82208
dc.description.abstract Cells inherit two centrioles, the older of which is uniquely capable of generating a cilium. Using proteomics and superresolved imaging, we identify a module that we term DISCO (distal centriole complex). The DISCO components CEP90, MNR, and OFD1 underlie human ciliopathies. This complex localizes to both distal centrioles and centriolar satellites, proteinaceous granules surrounding centrioles. Cells and mice lacking CEP90 or MNR do not generate cilia, fail to assemble distal appendages, and do not transduce Hedgehog signals. Disrupting the satellite pools does not affect distal appendage assembly, indicating that it is the centriolar populations of MNR and CEP90 that are critical for ciliogenesis. CEP90 recruits the most proximal known distal appendage component, CEP83, to root distal appendage formation, an early step in ciliogenesis. In addition, MNR, but not CEP90, restricts centriolar length by recruiting OFD1. We conclude that DISCO acts at the distal centriole to support ciliogenesis by restraining centriole length and assembling distal appendages, defects in which cause human ciliopathies.
dc.language.iso eng
dc.relation.ispartof Journal of Cell Biology, 2021, vol. 220, num. 9, p. 1-21
dc.rights.uri info:eu-repo/semantics/openAccess
dc.source Kumar, Dhivya Rains, Addison Herranz Pérez, Vicente Qualong, Lu Xiaoyu, Shi Swaney, Danielle L. Stevenson, Erika Krogan, Nevan J. Huang, Bo Westlake, Christopher García Verdugo, José Manuel Yoder, Bradley K. Reiter, Jeremy F. 2021 A ciliopathy complex builds distal appendages to initiate ciliogenesis Journal of Cell Biology 220 9 1 21
dc.subject Bioquímica
dc.title A ciliopathy complex builds distal appendages to initiate ciliogenesis
dc.type info:eu-repo/semantics/article
dc.date.updated 2022-04-07T09:45:45Z
dc.identifier.doi https://doi.org/10.1083/jcb.202011133
dc.identifier.idgrec 151251

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