Super-resolution architecture of mammalian centriole distal appendages reveals distinct blade and matrix functional components

Copyright © The Author(s) 2018. Distal appendages (DAPs) are nanoscale, pinwheel-like structures protruding from the distal end of the centriole that mediate membrane docking during ciliogenesis, marking the cilia base around the ciliary gate. Here we determine a super-resolved multiplex of 16 centriole-distal-end components. Surprisingly, rather than pinwheels, intact DAPs exhibit a cone-shaped architecture with components filling the space between each pinwheel blade, a new structural element we term the distal appendage matrix (DAM). Specifically, CEP83, CEP89, SCLT1, and CEP164 form the backbone of pinwheel blades, with CEP83 confined at the root and CEP164 extending to the tip near the membrane-docking site. By contrast, FBF1 marks the distal end of the DAM near the ciliary membrane. Strikingly, unlike CEP164, which is essential for ciliogenesis, FBF1 is required for ciliary gating of transmembrane proteins, revealing DAPs as an essential component of the ciliary gate. Our findings redefine both the structure and function of DAPs.Ministry of Science and Technology, Taiwan (Grant Number 103-2112-M-001-039-MY3); Academia Sinica Career Development Award, Academia Sinica Nano Program; University of Alabama at Birmingham (UAB) Hepato/Renal Fibrocystic Diseases Core Center (HRFDCC) Pilot Award (NIH 5P30DK074038-09); NIH grant GM088253, American Cancer Society grant RSG-14-153-01-CCG; Geoffrey Beene Cancer Research Center grant