50 Kowalski ML, Pawliczak R, Woźniak J, Siuda K, Grzegorczyk J, Pietrzak M, Kozłowski Z, Marek K (1998) Culture of human nasal epithelial cells from nasal polyps on collagen matrix. Arch Immunol Ther Exp 46(1):51–57 Lechner JF, LaVeck MA (1985) A serum-free method for culturing normal human bronchial epithelial cells at clonal density. J Tissue Culture Methods 9(2):43–48 Liu Z, Lu X, Wang H, You XJ, Gao QX, Cui YH (2007) Group II subfamily secretory phospholipase a2 enzymes: expression in chronic rhinosinusitis with and without nasal polyps. Allergy 62 (9):999–1006 Loges NT, Olbrich H, Fenske L, Mussaffi H, Horvath J, Fliegauf M, Kuhl H, Baktai G, Peterffy E, Chodhari R, Chung EM, Rutman A, O’Callaghan C, Blau H, Tiszlavicz L, Voelkel K, Witt M, Zietkiewicz E, Neesen J, Reinhardt R, Mitchison HM, Omran H (2008) Dnai2 mutations cause primary ciliary dyskinesia with defects in the outer dynein arm. Am J Hum Genet 83(5):547–558 Lopez-Souza N, Avila PC, Widdicombe JH (2003) Polarized cultures of human airway epithelium from nasal scrapings and bronchial brushings. In Vitro Cell Dev Biol Anim 39(7):266–269. doi:10.1290/1543-706X(2003)039<0266:PCOHAE>2.0.CO;2 Masui T, Wakefield LM, Lechner JF, LaVeck MA, Sporn MB, Harris CC (1986) Type beta transforming growth factor is the primary differentiation-inducing serum factor for normal human bronchial epithelial cells. Proc Natl Acad Sci USA 83(8):2438–2442 Meeks M, Bush A (2000) Primary ciliary dyskinesia (PCD). Pediatr Pulmonol 29(4):307–316 Moore A, Escudier E, Roger G, Tamalet A, Pelosse B, Marlin S, Clement A, Geremek M, Delaisi B, Bridoux AM, Coste A, Witt M, Duriez B, Amselem S (2006) Rpgr is mutated in patients with a complex x linked phenotype combining primary ciliary dyskinesia and retinitis pigmentosa. J Med Genet 43(4):326–333 Morillas HN, Zariwala M, Knowles MR (2007) Genetic causes of bronchiectasis: primary ciliary dyskinesia. Respiration 74 (3):252–263 Mygind N, Dahl R (1998) Anatomy, physiology and function of the nasal cavities in health and disease. Adv Drug Deliv Rev 29(1–2): 3–12 Neugebauer P, Endepols H, Mickenhagen A, Walger M (2003) Ciliogenesis in submersion and suspension cultures of human nasal epithelial cells. Eur Arch Otorhinolaryngol 260(6):325– 330. doi:10.1007/s00405-002-0562-y Noone PG, Leigh MW, Sannuti A, Minnix SL, Carson JL, Hazucha M, Zariwala MA, Knowles MR (2004) Primary ciliary dyskinesia: diagnostic and phenotypic features. Am J Respir Crit Care Med 169 (4):459–467 Olbrich H, Horváth J, Fekete A, Loges NT, Storm van’s Gravesande K, Blum A, Hörmann K, Omran H (2006) Axonemal localization of the dynein component DNAH5 is not altered in secondary ciliary dyskinesia. Pediatr Res 59(3):418–422 Omran H, Kobayashi D, Olbrich H, Tsukahara T, Loges NT, Hagiwara H, Zhang Q, Leblond G, O’Toole E, Hara C, Mizuno H, Kawano H, Fliegauf M, Yagi T, Koshida S, Miyawaki A, Zentgraf H, Seithe H, Reinhardt R, Watanabe Y, Kamiya R, Mitchell DR, Takeda H (2008) Ktu/PF13 is required for cytoplasmic pre- assembly of axonemal dyneins. Nature 456(7222):611–616 Ooi EH, Wormald PJ, Carney AS, James CL, Tan LW (2007) Surfactant protein D expression in chronic rhinosinusitis patients and immune responses in vitro to Aspergillus and Alternaria in a nasal explant model. Laryngoscope 117(1):51–57 Pennarun G, Escudier E, Chapelin C, Bridoux AM, Cacheux V, Roger G, Clément A, Goossens M, Amselem S, Duriez B (1999) Loss-of- function mutations in a human gene related to Chlamydomonas reinhardtii dynein IC78 result in primary ciliary dyskinesia. Am J Hum Genet 65(6):1508–1519 J Appl Genetics (2011) 52:39–51 Pifferi M, Cangiotti AM, Ragazzo V, Baldini G, Cinti S, Boner AL (2001) Primary ciliary dyskinesia: diagnosis in children with inconclusive ultrastructural evaluation. Pediatr Allergy Immunol 12(5):274–282. doi:10.1046/j.0905-6157.2001.00000.x Pifferi M, Montemurro F, Cangiotti AM, Ragazzo V, Di Cicco M, Vinci B, Vozzi G, Macchia P, Boner AL (2009) Simplified cell culture method for the diagnosis of atypical primary ciliary dyskinesia. Thorax 64(12):1077–1081. doi:10.1136/thx.2008.110940 Randell SH (2006) Airway epithelial stem cells and the pathophys- iology of chronic obstructive pulmonary disease. Proc Am Thorac Soc 3(8):718–725 Rearick JI, Jetten AM (1989) Effect of substratum and retinoids upon the mucosecretory differentiation of airway epithelial cells in vitro. Environ Health Perspect 80:229–237 Rock JR, Onaitis MW, Rawlins EL, Lu Y, Clark CP, Xue Y, Randell SH, Hogan BL (2009) Basal cells as stem cells of the mouse trachea and human airway epithelium. Proc Natl Acad Sci USA 106(31):12771–12775 Rutland J, de Iongh RU (1990) Random ciliary orientation. A cause of respiratory tract disease. N Engl J Med 323(24):1681–1684 Sachs LA, Finkbeiner WE, Widdicombe JH (2003) Effects of media on differentiation of cultured human tracheal epithelium. In Vitro Cell Dev Biol Anim 39(1–2):56–62. doi:10.1290/1543-706X (2003)039<0056:EOMODO>2.0.CO;2 Schidlow DV (1994) Primary ciliary dyskinesia (the immotile cilia syndrome). Ann Allergy 73(6):457–468, quiz 468–470 Schmidt MC, Peter H, Lang SR, Ditzinger G, Merkle HP (1998) In vitro cell models to study nasal mucosal permeability and metabolism. Adv Drug Deliv Rev 29(1–2):51–79. doi:10.1016/ S0169-409X(97)00061-6 Schwabe GC, Hoffmann K, Loges NT, Birker D, Rossier C, de Santi MM, Olbrich H, Fliegauf M, Failly M, Liebers U, Collura M, Gaedicke G, Mundlos S, Wahn U, Blouin JL, Niggemann B, Omran H, Antonarakis SE, Bartoloni L (2008) Primary ciliary dyskinesia associated with normal axoneme ultrastructure is caused by dnah11 mutations. Hum Mutat 29 (2):289–298 Smallman LA, Gregory J (1986) Ultrastructural abnormalities of cilia in the human respiratory tract. Hum Pathol 17(8):848–855 Steele VE, Arnold JT (1985) Isolation and long-term culture of rat, rabbit, and human nasal turbinate epithelial cells. In Vitro Cell Dev Biol 21(12):681–687 Van Scott MR, Lee NP, Yankaskas JR, Boucher RC (1988) Effect of hormones on growth and function of cultured canine tracheal epithelial cells. Am J Physiol 255(2 Pt 1):C237–C245 Werner U, Kissel T (1995) Development of a human nasal epithelial cell culture model and its suitability for transport and metabolism studies under in vitro conditions. Pharm Res 12(4):565–571 Whitcutt MJ, Adler KB, Wu R (1988) A biphasic chamber system for maintaining polarity of differentiation of cultured respiratory tract epithelial cells. In Vitro Cell Dev Biol 24(5):420–428 Wiesel JM, Gamiel H, Vlodavsky I, Gay I, Ben-Bassat H (1983) Cell attachment, growth characteristics and surface morphology of human upper-respiratory tract epithelium cultured on extracellular matrix. Eur J Clin Investig 13(1):57–63 Wilson R, Read R, Cole P (1992) Interaction of Haemophilus influenzae with mucus, cilia, and respiratory epithelium. J Infect Dis 165(Suppl 1):S100–S102 Wisseman CL, Simel DL, Spock A, Shelburne JD (1981) The prevalence of abnormal cilia in normal pediatric lungs. Arch Pathol Lab Med 105(10):552–555 Wu R, Yankaskas J, Cheng E, Knowles MR, Boucher R (1985) Growth and differentiation of human nasal epithelial cells in culture. Serum-free, hormone-supplemented medium and proteo- glycan synthesis. Am Rev Respir Dis 132(2):311–320