title: Specialized Cilia in Mammalian Sensory Systems.
abstract: Cilia and flagella are highly conserved and important microtubule-based organelles that project from the surface of eukaryotic cells and act as antennae to sense extracellular signals. Moreover, cilia have emerged as key players in numerous physiological, developmental, and sensory processes such as hearing, olfaction, and photoreception. Genetic defects in ciliary proteins responsible for cilia formation, maintenance, or function underlie a wide array of human diseases like deafness, anosmia, and retinal degeneration in sensory systems. Impairment of more than one sensory organ results in numerous syndromic ciliary disorders like the autosomal recessive genetic diseases Bardet-Biedl and Usher syndrome. Here we describe the structure and distinct functional roles of cilia in sensory organs like the inner ear, the olfactory epithelium, and the retina of the mouse. The spectrum of ciliary function in fundamental cellular processes highlights the importance of elucidating ciliopathy-related proteins in order to find novel potential therapies.
authors.affiliation: Department of Biology, Animal Physiology, University of Erlangen-Nuremberg, 91058 Erlangen, Germany. Nathalie.Falk@FAU.de.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Falk
authors.affiliation: Department of Biology, Animal Physiology, University of Erlangen-Nuremberg, 91058 Erlangen, Germany. Marlene.Loesl@FAU.de.
authors.forename: Marlene
authors.initials: M
authors.lastname: Lösl
authors.affiliation: Department of Biology, Animal Physiology, University of Erlangen-Nuremberg, 91058 Erlangen, Germany. Nadja.Schroeder@fau.de.
authors.forename: Nadja
authors.initials: N
authors.lastname: Schröder
authors.affiliation: Department of Biology, Animal Physiology, University of Erlangen-Nuremberg, 91058 Erlangen, Germany. Andreas.Giessl@FAU.de.
authors.forename: Andreas
authors.initials: A
authors.lastname: Gießl
keywords: connecting cilium; inner ear; intraflagellar transport; kinocilium; olfactory epithelium; primary cilia; retina