Proteins that control the geometry of microtubules at the ends of cilia.

The Journal of Cell Biology
Panagiota LoukaJacek Gaertig

Abstract

Cilia, essential motile and sensory organelles, have several compartments: the basal body, transition zone, and the middle and distal axoneme segments. The distal segment accommodates key functions, including cilium assembly and sensory activities. While the middle segment contains doublet microtubules (incomplete B-tubules fused to complete A-tubules), the distal segment contains only A-tubule extensions, and its existence requires coordination of microtubule length at the nanometer scale. We show that three conserved proteins, two of which are mutated in the ciliopathy Joubert syndrome, determine the geometry of the distal segment, by controlling the positions of specific microtubule ends. FAP256/CEP104 promotes A-tubule elongation. CHE-12/Crescerin and ARMC9 act as positive and negative regulators of B-tubule length, respectively. We show that defects in the distal segment dimensions are associated with motile and sensory deficiencies of cilia. Our observations suggest that abnormalities in distal segment organization cause a subset of Joubert syndrome cases.

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Citations

Feb 17, 2019·Cells·Helena SoaresJoão Gonçalves
May 6, 2019·Genes·Alejandro SaettoneJeffrey Fillingham
Jul 20, 2019·PloS One·Jonathan B Leano, Kevin C Slep
Oct 19, 2019·Molecular Genetics & Genomic Medicine·Minna LuoXu Ma
Nov 19, 2019·Frontiers in Cell and Developmental Biology·Brian A BaylessMark Winey
Oct 18, 2020·Developmental Neurobiology·Jyothi S Akella, Maureen M Barr
Apr 16, 2021·Current Biology : CB·Kwangjin ParkMichel R Leroux

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Datasets Mentioned

BETA
AAH01640.1

Methods Mentioned

BETA
transmission electron microscopy
electron microscopy
PCR
genotyping

Software Mentioned

TASSER
SR
ImageJ
regulation Data Harvester
Matlab
SIM
ClustalW
NIH ImageJ
Co
AxioVision

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