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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

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.citation	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.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.subject	Bioquímica
dc.title	A ciliopathy complex builds distal appendages to initiate ciliogenesis
dc.type	journal article	es_ES
dc.date.updated	2022-04-07T09:45:45Z
dc.identifier.doi	10.1083/jcb.202011133
dc.identifier.idgrec	151251
dc.accrualmethod
dc.rights.accessRights	open access	es_ES