Specialized Cilia in Mammalian Sensory Systems

Cells

Nathalie Falk²  Marlene Lösl²  Nadja Schrr²  Andreas Gießl¹  Gang Dong² 

[1] Department of Biology, Animal Physiology, University of Erlangen-Nuremberg, 91058 Erlangen, Germany;

关键词: primary cilia;    intraflagellar transport;    kinocilium;    inner ear;    olfactory epithelium;    retina;    connecting cilium;   

DOI  :  10.3390/cells4030500

来源: mdpi

【 摘 要 】

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.

【 授权许可】

CC BY   
© 2015 by the authors; licensee MDPI, Basel, Switzerland.