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

Autor:	Louka P; Department of Cellular Biology, University of Georgia, Athens, GA., Vasudevan KK; Department of Cellular Biology, University of Georgia, Athens, GA., Guha M; Department of Cellular Biology, University of Georgia, Athens, GA., Joachimiak E; Laboratory of Cytoskeleton and Cilia Biology, Nencki Institute of Experimental Biology of Polish Academy of Sciences, Warsaw, Poland., Wloga D; Laboratory of Cytoskeleton and Cilia Biology, Nencki Institute of Experimental Biology of Polish Academy of Sciences, Warsaw, Poland., Tomasi RF; Department of Mechanics, LadHyX, Ecole Polytechnique-Centre National de la Recherche Scientifique, Palaiseau, France., Baroud CN; Department of Mechanics, LadHyX, Ecole Polytechnique-Centre National de la Recherche Scientifique, Palaiseau, France., Dupuis-Williams P; UMR-S1174 Institut National de la Santé et de la Recherche Médicale, Université Paris-Sud, Bat 443, Orsay, France.; École Supérieure de Physique et de Chimie Industrielles de la Ville de Paris, Paris, France., Galati DF; Department of Cell and Developmental Biology, University of Colorado Anschutz Medical Campus, Aurora, CO., Pearson CG; Department of Cell and Developmental Biology, University of Colorado Anschutz Medical Campus, Aurora, CO., Rice LM; Departments of Biophysics and Biochemistry, University of Texas Southwestern Medical Center, Dallas, TX., Moresco JJ; Department of Molecular Medicine, The Scripps Research Institute, La Jolla, CA., Yates JR 3rd; Department of Molecular Medicine, The Scripps Research Institute, La Jolla, CA., Jiang YY; Department of Cellular Biology, University of Georgia, Athens, GA., Lechtreck K; Department of Cellular Biology, University of Georgia, Athens, GA., Dentler W; Department of Molecular Biosciences, University of Kansas, Lawrence, KS wdent@ku.edu., Gaertig J; Department of Cellular Biology, University of Georgia, Athens, GA jgaertig@uga.edu.
Jazyk:	angličtina
Zdroj:	The Journal of cell biology [J Cell Biol] 2018 Dec 03; Vol. 217 (12), pp. 4298-4313. Date of Electronic Publication: 2018 Sep 14.
DOI:	10.1083/jcb.201804141
Abstrakt:	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. (© 2018 Louka et al.)
Databáze:	MEDLINE
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