1.Gadsby DC, Vergani P, Csanady L: The ABC protein turned chloride channel whose failure causes cystic fibrosis. Nature 2006, 440(7083):477–483.
2.Tizzano EF, Chitayat D, Buchwald M: Cell-specific localization of CFTR mRNA shows developmentally regulated expression in human fetal tissues. Hum Mol Genet 1993, 2(3):219–224.
3.Riordan JR: CFTR function and prospects for therapy. Annu Rev Biochem 2008, 77:701–726.
4.Quinton PM: Physiological basis of cystic fibrosis: a historical perspective. Physiological reviews 1999, 79(1 Suppl):S3-S22.
5.Johannesson B, Hirtz S, Schatterny J, Schultz C, Mall MA: CFTR regulates early pathogenesis of chronic obstructive lung disease in betaENaC-overexpressing mice. PLoS One 2012, 7(8):e44059.
6.Pezzulo AA, Tang XX, Hoegger MJ, Alaiwa MH, Ramachandran S, Moninger TO, Karp PH, Wohlford-Lenane CL, Haagsman HP, van Eijk M et al: Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature 2012, 487(7405):109–113.
7.Wilschanski M, Novak I: The cystic fibrosis of exocrine pancreas. Cold Spring Harbor perspectives in medicine 2013, 3(5):a009746.
8.Guo JH, Chen H, Ruan YC, Zhang XL, Zhang XH, Fok KL, Tsang LL, Yu MK, Huang WQ, Sun X et al: Glucose-induced electrical activities and insulin secretion in pancreatic islet beta-cells are modulated by CFTR. Nature communications 2014, 5:4420.
9.Xu WM, Shi QX, Chen WY, Zhou CX, Ni Y, Rowlands DK, Yi Liu G, Zhu H, Ma ZG, Wang XF et al: Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility. Proc Natl Acad Sci U S A 2007, 104(23):9816–9821.
10.Lu YC, Chen H, Fok KL, Tsang LL, Yu MK, Zhang XH, Chen J, Jiang X, Chung YW, Ma AC et al: CFTR mediates bicarbonate-dependent activation of miR–125b in preimplantation embryo development. Cell Res 2012, 22(10):1453–1466.
11.Chen H, Guo JH, Lu YC, Ding GL, Yu MK, Tsang LL, Fok KL, Liu XM, Zhang XH, Chung YW et al: Impaired CFTR-dependent amplification of FSH-stimulated estrogen production in cystic fibrosis and PCOS. J Clin Endocrinol Metab 2012, 97(3):923–932.
12.Davies JC, Alton EW, Bush A: Cystic fibrosis. BMJ 2007, 335(7632):1255–1259.
13.Labombarda F, Saloux E, Brouard J, Bergot E, Milliez P: Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes? Respir Med 2016, 118:31–38.
14.Bright-Thomas RJ, Webb AK: The heart in cystic fibrosis. J R Soc Med 2002, 95 Suppl 41:2–10.
15.Ozcelik N, Shell R, Holtzlander M, Cua C: Decreased right ventricular function in healthy pediatric cystic fibrosis patients versus non-cystic fibrosis patients. Pediatr Cardiol 2013, 34(1):159–164.
16.Labombarda F, Pellissier A, Ellafi M, Creveuil C, Ribault V, Laurans M, Guillot M, Bergot E, Grollier G, Milliez P et al: Myocardial strain assessment in cystic fibrosis. J Am Soc Echocardiogr 2011, 24(9):1037–1045.
17.Sellers ZM, McGlocklin L, Brasch A: Strain rate echocardiography uncovers subclinical left ventricular dysfunction in cystic fibrosis. J Cyst Fibros 2015, 14(5):654–660.
18.Bakkers J: Zebrafish as a model to study cardiac development and human cardiac disease. Cardiovasc Res 2011, 91(2):279–288.
19.Sayyid ZN, Sellers ZM: Technological advances shed light on left ventricular cardiac disturbances in cystic fibrosis. J Cyst Fibros 2017, 16(4):454–464.
20.Westerfield M: The Zebrafish Book. Oregon: University of Oregon Press; 1993.
21.Kimmel CB, Ballard WW, Kimmel SR, Ullmann B, Schilling TF: Stages of embryonic development of the zebrafish. Dev Dyn 1995, 203(3):253–310.
22.Thisse C, Thisse B: High-resolution in situ hybridization to whole-mount zebrafish embryos. Nature protocols 2008, 3(1):59–69.
23.Sun H, Li D, Chen S, Liu Y, Liao X, Deng W, Li N, Zeng M, Tao D, Ma Y: Zili inhibits transforming growth factor-beta signaling by interacting with Smad4. J Biol Chem 2010, 285(6):4243–4250.
24.Liao H, Chen Y, Li Y, Xue S, Liu M, Lin Z, Liu Y, Chan HC, Zhang X, Sun H: CFTR is required for the migration of primordial germ cells during zebrafish early embryogenesis. Reproduction 2018, 156(3):261–268.
25.Sun H, Wang Y, Zhang J, Chen Y, Liu Y, Lin Z, Liu M, Sheng K, Liao H, Tsang KS et al: CFTR mutation enhances Dishevelled degradation and results in impairment of Wnt-dependent hematopoiesis. Cell Death Dis 2018, 9(3):275.
26.Navis A, Marjoram L, Bagnat M: Cftr controls lumen expansion and function of Kupffer’s vesicle in zebrafish. Development 2013, 140(8):1703–1712.
27.Ma T, Thiagarajah JR, Yang H, Sonawane ND, Folli C, Galietta LJ, Verkman AS: Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion. J Clin Invest 2002, 110(11):1651–1658.
28.Taddei A, Folli C, Zegarra-Moran O, Fanen P, Verkman AS, Galietta LJ: Altered channel gating mechanism for CFTR inhibition by a high-affinity thiazolidinone blocker. FEBS Lett 2004, 558(1–3):52–56.
29.Li H, Findlay IA, Sheppard DN: The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors. Kidney Int 2004, 66(5):1926–1938.
30.Ngiam NS, Chong SS, Shek LP, Goh DL, Ong KC, Chng SY, Yeo GH, Goh DY: Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study. J Cyst Fibros 2006, 5(3):159–164.
31.Sakamoto H, Yajima T, Suzuki K, Ogawa Y: Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation associated with a congenital bilateral absence of vas deferens. Int J Urol 2008, 15(3):270–271.
32.Lu S, Yang X, Cui Y, Li X, Zhang H, Liu J, Chen ZJ: Different cystic fibrosis transmembrane conductance regulator mutations in Chinese men with congenital bilateral absence of vas deferens and other acquired obstructive azoospermia. Urology 2013, 82(4):824–828.
33.Shen Y, Liu J, Zhong L, Mogayzel PJ, Jr., Zeitlin PL, Sosnay PR, Zhao S: Clinical Phenotypes and Genotypic Spectrum of Cystic Fibrosis in Chinese Children. J Pediatr 2016, 171:269–276 e261.
34.Lee JH, Choi JH, Namkung W, Hanrahan JW, Chang J, Song SY, Park SW, Kim DS, Yoon JH, Suh Y et al: A haplotype-based molecular analysis of CFTR mutations associated with respiratory and pancreatic diseases. Hum Mol Genet 2003, 12(18):2321–2332.
35.Amaral MD: CFTR and chaperones: processing and degradation. Journal of molecular neuroscience: MN 2004, 23(1–2):41–48.
36.Welsh MJ, Smith AE: Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 1993, 73(7):1251–1254.
37.Hwang TC, Kirk KL: The CFTR ion channel: gating, regulation, and anion permeation. Cold Spring Harbor perspectives in medicine 2013, 3(1):a009498.
38.Ryland D, Reid L: The pulmonary circulation in cystic fibrosis. Thorax 1975, 30(3):285–292.
39.Berger M: Inflammatory mediators in cystic fibrosis lung disease. Allergy Asthma Proc 2002, 23(1):19–25.
40.Torriani R, Chassot J, Stocker F, Weber JW: Two children with cystic fibrosis and myocardial fibrosis. Monogr Paediatr 1979, 10:155–157.
41.Wiebicke W, Artlich A, Gerling I: Myocardial fibrosis—a rare complication in patients with cystic fibrosis. Eur J Pediatr 1993, 152(8):694–696.
42.Cheron G, Paradis K, Steru D, Demay G, Lenoir G: Cardiac involvement in cystic fibrosis revealed by a ventricular arrhythmia. Acta Paediatr Scand 1984, 73(5):697–700.
43.Zimmermann A, Stocker F, Johr M, Torriani R, Chassot J, Weber JW: Cardiomyopathy in cystic fibrosis: lymphoedema of the heart with focal myocardial fibrosis. Helv Paediatr Acta 1982, 37(2):183–192.
44.Stern RC, Borkat G, Hirschfeld SS, Boat TF, Matthews LW, Liebman J, Doershuk CF: Heart failure in cystic fibrosis. Treatment and prognosis of cor pulmonale with failure of the right side of the heart. Am J Dis Child 1980, 134(3):267–272.
45.Lakdawala NK, Winterfield JR, Funke BH: Dilated cardiomyopathy. Circ Arrhythm Electrophysiol 2013, 6(1):228–237.
46.Reddy MM, Quinton PM: Functional interaction of CFTR and ENaC in sweat glands. Pflugers Arch 2003, 445(4):499–503.