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2015 ; 4
(3
): 500-19
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Specialized Cilia in Mammalian Sensory Systems
#MMPMID26378583
Falk N
; Lösl M
; Schröder N
; Gießl A
Cells
2015[Sep]; 4
(3
): 500-19
PMID26378583
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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.
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