Abstract
Cilia are extensions of the apical membranes. The cilium itself is characterized by a 9 + 2 axonemal structure. An active, coordinated ciliary beating is essential for mucociliary transport. Ciliary beating depends on the ATPase activity in the dynein arms and is characterized by a specific beating pattern. In healthy persons, 95 % of the cilia are ultrastructurally completely normal. Ciliary abnormalities can be the results of external factors (secondary ciliary dyskinesia) or inherited (primary ciliary dyskinesia). Ciliary function and structure are organized at different levels from the individual cilia, over interciliary and intercellular interaction, to the macroscopic level of the ciliated tapestry and mucociliary transport.
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Afzelius BA. The immotile-cilia syndrome: a microtubule-associated defect. CRC Crit Rev Biochem. 1985;19:63–87.
Barbato A, Frischer T, Kuehni CE, et al. Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children. Eur Respir J. 2009;34:1264–76.
Becker-Heck A, Zohn IE, Okabe N, et al. The coiled-coil domain containing protein CCDC40 is essential for motile cilia function and left-right axis formation. Nat Genet. 2011;43:79–84.
Castleman VH, Romio L, Chodhari R, Hirst RA, et al. Mutations in radial spoke head protein genes RSPH9 and RSPH4A cause primary ciliary dyskinesia with central-microtubular-pair abnormalities. Am J Hum Genet. 2009;84:197–209.
Chilvers MA, Rutman A, O’Callaghan C. Ciliary beat pattern is associated with specific ultrastructural defects in primary ciliary dyskinesia. J Allergy Clin Immunol. 2003;112:518–24.
Ferkol TW, Leigh MW. Ciliopathies: the central role of cilia in a spectrum of pediatric disorders. J Pediatr. 2012;160(3):366–71.
Hornef N, Olbrich H, Horvath J, Zariwala MA, et al. DNAH5 mutations are a common cause of primary ciliary dyskinesia with outer dynein arm defects. Am J Respir Crit Care Med. 2006;174:120–6.
Ingels K, Kortmann M, Nijziel M, et al. Factors influencing ciliary beat measurements. Rhinology. 1991;29:19–26.
Jafek BW. Ultrastructure of human nasal mucosa. Laryngoscope. 1983;93:1576–99.
Jorissen M. Correlations among mucociliary transport, ciliary function and ciliary structure. Am J Rhinol. 1998;12:53–8.
Jorissen M, Willems T. The secondary nature of ciliary (dis)orientation in secondary and primary ciliary dyskinesia. Acta Otolaryngol. 2004;124:527–31.
Jorissen M, De Brouwer J, Bessems A, Cassiman JJ. Quantitation of ciliary beat frequency by computerized microscope photometry – a preliminary study on suspension cultures of human nasal epithelia showing spontaneous ciliogenesis in vitro. Leitz Sci Tech Info. 1992;10:88–93.
Kim CS, Jeon SY, Min YG, et al. Effects of beta-toxin of Staphylococcus aureus on ciliary activity of nasal epithelial cells. Laryngoscope. 2000;110:2085–8.
King SM. Axonemal dyneins winch the cilium. Nat Struct Mol Biol. 2010;17:673–4.
Knowles MR, Boucher RC. Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest. 2002;109:571–7.
Knowles MR, Leigh MW, Carson JL, et al. Mutations of DNAH11 in patients with primary ciliary dyskinesia with normal ciliary ultrastructure. Thorax. 2012;67:433–41.
Loges NT, Olbrich H, Fenske L, et al. DNAI2 mutations cause primary ciliary dyskinesia with defects in the outer dynein arm. Am J Hum Genet. 2008;83:547–58.
Mallants R, Jorissen M, Augustijns P. Beneficial effect of antibiotics on ciliary beat frequency of human nasal epithelial cells exposed to bacterial toxins. J Pharm Pharmacol. 2008;60:437–43.
Mallik R, Carter BC, Lex SA, et al. Cytoplasmic dynein functions as a gear in response to load. Nature. 2004;427:649–52.
Mazor M, Alkrinawi S, Chalifa-Caspi V, et al. Primary ciliary dyskinesia caused by homozygous mutation in DNAL1, encoding dynein light chain 1. Am J Hum Genet. 2011;88:599–607.
Melville GN, Horstmann G, Iravani J. Adrenergic compounds and the respiratory tract. A physiological and electron-microscopical study. Respiration. 1976;33:261–9.
Merkus F, Verhoef J, Schipper N, et al. Nasal mucociliary clearance as a factor in nasal drug delivery. Adv Drug Deliv Rev. 1998;29:13–38.
Pazour GJ, Agrin N, Walker BL, et al. Identification of predicted human outer dynein arm genes: candidates for primary ciliary dyskinesia genes. J Med Genet. 2006;43:62–73.
Rautiainen M, Collan Y, Nuutinen J. A method for measuring the orientation (beat direction) of respiratory cilia. Arch Otorhinolaryngol. 1986;243:265–8.
Rautiainen M, Collan Y, Nuutinen J, et al. Ciliary orientation in the ‘immotile cilia’ syndrome. Eur Arch Otorhinolaryngol. 1990;247:100–3.
Rayner CF, Rutman A, Dewar A, et al. Ciliary disorientation alone as a cause of primary ciliary dyskinesia syndrome. Am J Respir Crit Care Med. 1996;153:1123–9.
Rhodin JAG. Ultrastructure and function of human tracheal mucosa. Am Rev Respir Dis. 1966;93:1–15.
Roberts AJ, Numata N, Walker ML, et al. AAA + Ring and linker swing mechanism in the dynein motor. Cell. 2009;136:485–95.
Sackner MA, Epstein S, Wanner A. Effect of beta-adrenergic agonists aerosolized by freon propellant on tracheal mucous velocity and cardiac output. Chest. 1976;69:593–8.
Sakakibara H, Kamiya R. Functional recombination of outer dynein arms with outer arm-missing flagellar axonemes of a Chlamydomonas mutant. J Cell Sci. 1989;92:77–83.
Sanderson MJ, Chow I, Dirksen ER. Intercellular communication between ciliated cells in culture. Am J Physiol. 1988;254:C63–74.
Satir P. How cilia move. Sci Am. 1974;231:44–52.
Satir P, Christensen ST. Overview of structure and function of mammalian cilia. Annu Rev Physiol. 2007;69:377–400.
Schmid A, Salathe M. Ciliary beat co-ordination by calcium. Biol Cell. 2011;103:159–69.
van de Donk HJ, Zuidema J, Merkus FW. The influence of the pH and osmotic pressure upon tracheal ciliary beat frequency as determined with a new photo-electric registration device. Rhinology. 1980;18:93–104.
Verdugo P, Johnson NT, Tam PY. Beta-Adrenergic stimulation of respiratory ciliary activity. J Appl Physiol. 1980;48:868–71.
Wong LB, Miller IF, Yeates DB. Nature of the mammalian ciliary metachronal wave. J Appl Physiol. 1993;75:458–67.
Wood RE, Wanner A, Hirsch J, Farrell PM. Tracheal mucociliary transport in patients with cystic fibrosis and its stimulation by terbutaline. Am Rev Respir Dis. 1975;111:733–8.
Yun YS, Min YG, Rhee CS, et al. Effects of alpha-toxin of Staphylococcus aureus on the ciliary activity and ultrastructure of human nasal ciliated epithelial cells. Laryngoscope. 1999;109:2021–4.
Zariwala MA, Leigh MW, Ceppa F, et al. Mutations of DNAI1 in primary ciliary dyskinesia: evidence of founder effect in a common mutation. Am J Respir Crit Care Med. 2006;174:858–66.
Zariwala MA, Omran H, Ferkol TW. The emerging genetics of primary ciliary dyskinesia. Proc Am Thorac Soc. 2011;8:430–3.
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Jorissen, M., Jaspers, M. (2013). Cilia, Ciliary Movement, and Mucociliary Transport. In: Önerci, T. (eds) Nasal Physiology and Pathophysiology of Nasal Disorders. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-37250-6_2
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DOI: https://doi.org/10.1007/978-3-642-37250-6_2
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