Nearly every cell in the human body has one or more protrusive structures called cilia or flagella. These power cell movement and fluid flow, sense the extracellular environment, coordinate cell signaling, and establish left-right asymmetry during development. Mutations in genes that encode cilia can lead to disorders known as ciliopathies.
Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Biology examines key aspects of ciliary biology-from the molecular to the organismal level-in normal physiology and disease. The contributors dissect the complex structures of motile and nonmotile (primary) cilia, discuss how the intraflagellar transport machinery moves cargo across the central axoneme, and review how the ciliary gate controls the composition of cilia and flagella. The roles of cilia in coordinating cellular responses to environmental stimuli via cell signaling pathways (e.g., Hedgehog) are also covered, as are physiological functions in processes such as fertilization, mucociliary clearance, and vision.
The authors also survey the wide spectrum of ciliopathies, describing their genetic bases, pathogenic mechanisms, and clinical manifestations. This volume is therefore an indispensable reference for all cell and developmental biologists, as well as medical geneticists and clinical scientists wishing to understand and treat disorders involving ciliary dysfunction.
Contents Preface Axoneme Structure from Motile Cilia Takashi Ishikawa Post-Translational Modifications of Tubulin and Cilia Dorota Wloga, Ewa Joachimiak, Panagiota Louka, and Jacek Gaertig Axonemal Dynein Arms Stephen M. King The Central Apparatus of Cilia and Eukaryotic Flagella Thomas D. Loreng and Elizabeth F. Smith Radial Spokes--A Snapshot of the Motility Regulation, Assembly, and Evolution of Cilia and Flagella Xiaoyan Zhu, Yi Liu, and Pinfen Yang The Intraflagellar Transport Machinery Michael Taschner and Esben Lorentzen Open Sesame: How Transition Fibers and the Transition Zone Control Ciliary Composition Francesc R. Garcia-Gonzalo and Jeremy F. Reiter Transition Zone Migration, a Mechanism for Cytoplasmic Ciliogenesis and Post- Axonemal Centriole Elongation Tomer Avidor-Reiss, Andrew Ha, and Marcus L. Basiri Primary Cilia and Coordination of Receptor Tyrosine Kinase (RTK) and Transforming Growth Factor beta (TGFß) Signaling Søren T. Christensen, Stine K. Morthorst, Johanne B. Morgensen, and Lotte B. Pedersen Primary Cilia and Mammalian Hedgehog Signaling Fiona Bangs and Kathryn V. Anderson G Protein-Coupled Receptor Signaling in Cilia Kirk Mykytyn and Candice Askwith Multiciliated Cells in Animals Alice Meunier and Juliette Azimzadeh Cilia in Left-Right Symmetry Breaking Kyosuke Shinohara and Hiroshi Hamada Ciliopathies Daniela A. Braun and Friedhelm Hildebrandt Ciliary Mechanisms of Cyst Formation in Polycystic Kidney Disease Ming Ma, Anna-Rachel Gallagher, and Stefan Somlo Cilia and Obesity Christian Vaisse, Jeremy F. Reiter, and Nicolas F. Berbari Sperm Sensory Signaling Dagmar Wachten, J.F. Jikeli, and U. Benjamin Kaupp Cilia and Mucociliary Clearance Ximena M. Bustamante-Marin and Lawrence E. Ostrowski Discovery, Diagnosis, and Etiology of Craniofacial Ciliopathies Elizabeth N. Schock and Samantha A. Brugmann Cilia and Ciliopathies in Congenital Heart Disease Nikolai T. Klena, Brian C. Gibbs, and Cecilia W. Lo Photoreceptor Cilia and Retinal Ciliopathies Kinga M. Bujakowski, Qin Liu, and Eric A. Pierce Evolution of Cilia David R. Mitchell Index