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© Research
Publication : The Journal of cell biology

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

Scientific Fields
Diseases
Organisms
Applications
Technique

Published in The Journal of cell biology - 14 Sep 2018

Louka P, Vasudevan KK, Guha M, Joachimiak E, Wloga D, Tomasi RF, Baroud CN, Dupuis-Williams P, Galati DF, Pearson CG, Rice LM, Moresco JJ, Yates JR, Jiang YY, Lechtreck K, Dentler W, Gaertig J

Link to Pubmed [PMID] – 30217954

J. Cell Biol. 2018 Sep;

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.

https://www.ncbi.nlm.nih.gov/pubmed/30217954