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. Cilia 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.
Preface
Axoneme Structure from Motile Cilia
Takashi Ishikawa
Posttranslational 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 Postaxonemal Centriole Elongation
Tomer Avidor-Reiss, Andrew Ha, and Marcus L. Basiri
Primary Cilia and Coordination of Receptor Tyrosine Kinase (RTK) and Transforming Growth Factor ß (TGF-ß) Signaling
Søren T. Christensen, Stine K. Morthorst, Johanne B. Mogensen, 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, Jan 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. Bujakowska, Qin Liu, and Eric A. Pierce
Evolution of Cilia
David R. Mitchell
Index