The long and winding road: stem cells for cystic fibrosis

References

• Cystic Fibrosis Mutation Database. 2017 [2017 Sept 10]. Available from: http://www.genet.sickkids.on.ca.
   Google Scholar
• Sosnay PR, Siklosi KR, van Goor F, et al. Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet. 2013 Oct;45(10):1160–1167.
   PubMed Web of Science ®Google Scholar
• Quinton PM. Physiological basis of cystic fibrosis: a historical perspective. Physiol Rev. 1999;79(1 Suppl):S3–S22.
   PubMedGoogle Scholar
• Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005;352(19):1992–2001.
   PubMed Web of Science ®Google Scholar
• Chan HC, Ruan YC, He Q, et al. The cystic fibrosis transmembrane conductance regulator in reproductive health and disease. J Physiol. 2009;587(Pt 10):2187–2195.
   PubMed Web of Science ®Google Scholar
• Amaral MD. Novel personalized therapies for cystic fibrosis: treating the basic defect in all patients. J Intern Med. 2015;277(2):155–166.
   PubMed Web of Science ®Google Scholar
• Bobadilla JL, Macek M Jr., Fine JP, et al. Cystic fibrosis: a worldwide analysis of CFTR mutations–correlation with incidence data and application to screening. Hum Mutat. 2002;19(6):575–606.
   PubMed Web of Science ®Google Scholar
• Wilschanski M, Novak I. The cystic fibrosis of exocrine pancreas. Cold Spring Harb Perspect Med. 2013;3(5):a009746.
   PubMed Web of Science ®Google Scholar
• Rowentree RK, Harris A. The phenotypic consequences of CFTR mutations. Ann Hum Genet. 2003;67:471–485.
   PubMed Web of Science ®Google Scholar
• Cohn JA, Strong TV, Picciotto MR, et al. Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells. Gastroenterology. 1993;105(6):1857–1864.
   PubMed Web of Science ®Google Scholar
• Kinnman N, Lindblad A, Housset C, et al. Expression of cystic fibrosis transmembrane conductance regulator in liver tissue from patients with cystic fibrosis. Hepatology. 2000;32(2):334–340.
   PubMed Web of Science ®Google Scholar
• Diwakar V, Pearson L, Beath S. Liver disease in children with cystic fibrosis. Paediatr Respir Rev. 2001;2(4):340–349.
   PubMedGoogle Scholar
• Gibson-Corley KN, Meyerholz DK, Engelhardt JF. Pancreatic pathophysiology in cystic fibrosis. J Pathol. 2016;238(2):311–320.
   PubMed Web of Science ®Google Scholar
• Li L, Somerset S. Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy. Dig Liver Dis. 2014;46(10):865–874.
   PubMedGoogle Scholar
• Boucher RC. Evidence for airway surface dehydration as the initiating event in CF airway disease. J Intern Med. 2007;261(1):5–16.
   PubMed Web of Science ®Google Scholar
• Bruscia EM, Bonfield TL. Innate and adaptive immunity in cystic fibrosis. Clin Chest Med. 2016;37(1):17–29.
   PubMed Web of Science ®Google Scholar
• Sly PD, Gangell CL, Chen L, et al. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013;368(21):1963–1970.
   PubMed Web of Science ®Google Scholar
• Adler FR, Aurora P, Barker DH, et al. Lung transplantation for cystic fibrosis. Proc Am Thorac Soc. 2009;6(8):619–633.
   PubMedGoogle Scholar
• Gaskin KJ. Nutritional care in children with cystic fibrosis: are our patients becoming better? Eur J Clin Nutr. 2013;67(5):558–564.
   PubMed Web of Science ®Google Scholar
• Chmiel JF, Konstan MW, Elborn JS. Antibiotic and anti-inflammatory therapies for cystic fibrosis. Cold Spring Harb Perspect Med. 2013;3(10):a009779.
   PubMed Web of Science ®Google Scholar
• Kuk K, Taylor-Cousar JL. Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects. Ther Adv Respir Dis. 2015;9(6):313–326.
   PubMed Web of Science ®Google Scholar
• Dhooghe B, Haaf JB, Noel S, et al. Strategies in early clinical development for the treatment of basic defects of cystic fibrosis. Expert Opin Investig Drugs. 2016;25(4):423–436.
   PubMed Web of Science ®Google Scholar
• Cohen D, Raftery J. Paying twice: questions over high cost of cystic fibrosis drug developed with charitable funding. Bmj. 2014;348:g1445.
   PubMed Web of Science ®Google Scholar
• Patel S, Sinha IP, Dwan K, et al. Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis. Cochrane Database Syst Rev. 2015(3):CD009841.
   Google Scholar
• Clancy JP, Rowe SM, Accurso FJ, et al. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax. 2012;67(1):12–18.
   PubMed Web of Science ®Google Scholar
• Wainwright CE, Elborn JS, Ramsey BW, et al. Lumacaftor-Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373(3):220–231.
   PubMed Web of Science ®Google Scholar
• ClinicalTrials.gov. 2017. [cited 2017 Nov 12]. Available from: https://clinicaltrials.gov.
   Google Scholar
• Cystic Fibrosis News Today. 2017. Data from Vertex’s triple-combination trials show promising benefits for CF patients; [cited 2017 Nov 13]. Available from: https://cysticfibrosisnewstoday.com/2017/07/19/cf-patients-see-benefits-from-vertexs-triple-combination-trials-for-patients-with-minimal-function-mutations/.
   Google Scholar
• Griesenbach U, Alton EW. Expert opinion in biological therapy: update on developments in lung gene transfer. Expert Opin Biol Ther. 2013;13(3):345–360.
   PubMed Web of Science ®Google Scholar
• Beers MF, Morrisey EE. The three R’s of lung health and disease: repair, remodeling, and regeneration. J Clin Invest. 2011;121(6):2065–2073.
   PubMed Web of Science ®Google Scholar
• Wansleeben C, Barkauskas CE, Rock JR, et al. Stem cells of the adult lung: their development and role in homeostasis, regeneration, and disease. Wiley Interdiscip Rev Dev Biol. 2013;2(1):131–148.
   PubMed Web of Science ®Google Scholar
• Kotton DN, Morrisey EE. Lung regeneration: mechanisms, applications and emerging stem cell populations. Nat Med. 2014;20(8):822–832.
   PubMed Web of Science ®Google Scholar
• Akram KM, Patel N, Spiteri MA, et al. Lung regeneration: endogenous and exogenous stem cell mediated therapeutic approaches. Int J Mol Sci. 2016;17(1).
   PubMed Web of Science ®Google Scholar
• Weiss DJ, Berberich MA, Borok Z, et al. Adult stem cells, lung biology, and lung disease. NHLBI/Cystic fibrosis foundation workshop. Proc Am Thorac Soc. 2006;3(3):193–207.
   PubMedGoogle Scholar
• Lau AN, Goodwin M, Kim CF, et al. Stem cells and regenerative medicine in lung biology and diseases. Mol Ther. 2012;20(6):1116–1130.
   PubMed Web of Science ®Google Scholar
• Weiss DJ, Chambers D, Giangreco A, et al. An official American Thoracic Society workshop report: stem cells and cell therapies in lung biology and diseases. Ann Am Thorac Soc. 2015;12(4):S79–97.
   PubMed Web of Science ®Google Scholar
• Summer R, Fitzsimmons K, Dwyer D, et al. Isolation of an adult mouse lung mesenchymal progenitor cell population. Am J Respir Cell Mol Biol. 2007;37(2):152–159.
   PubMed Web of Science ®Google Scholar
• Martin J, Helm K, Ruegg P, et al. Adult lung side population cells have mesenchymal stem cell potential. Cytotherapy. 2008;10(2):140–151.
   PubMed Web of Science ®Google Scholar
• Hoffman AM, Paxson JA, Mazan MR, et al. Lung-derived mesenchymal stromal cell post-transplantation survival, persistence, paracrine expression, and repair of elastase-injured lung. Stem Cells Dev. 2011;20(10):1779–1792.
   PubMed Web of Science ®Google Scholar
• Leeman KT, Fillmore CM, Kim CF. Lung stem and progenitor cells in tissue homeostasis and disease. Curr Top Dev Biol. 2014;107:207–233.
   PubMed Web of Science ®Google Scholar
• Alapati D, Morrisey EE. Gene editing and genetic lung disease. Basic research meets therapeutic application. Am J Respir Cell Mol Biol. 2017;56(3):283–290.
   PubMed Web of Science ®Google Scholar
• Zhang XH, Tee LY, Wang XG, et al. Off-target effects in CRISPR/Cas9-mediated genome engineering. Mol Ther Nucleic Acids. 2015;4:e264.
   PubMedGoogle Scholar
• Takahashi K, Yamanaka S. Induction of pluripotent stem cells from mouse embryonic and adult fibroblast cultures by defined factors. Cell. 2006;126(4):663–676.
   PubMed Web of Science ®Google Scholar
• Takahashi K, Tanabe K, Ohnuki M, et al. Induction of pluripotent stem cells from adult human fibroblasts by defined factors. Cell. 2007;131(5):861–872.
   PubMed Web of Science ®Google Scholar
• Coraux C, Nawrocki-Raby B, Hinnrasky J, et al. Embryonic stem cells generate airway epithelial tissue. Am J Respir Cell Mol Biol. 2005;32(2):87–92.
   PubMed Web of Science ®Google Scholar
• Rippon HJ, Lane S, Qin M, et al. Embryonic stem cells as a source of pulmonary epithelium in vitro and in vivo. Proc Am Thorac Soc. 2008;5(6):717–722.
   PubMedGoogle Scholar
• McIntyre BA, Alev C, Mechael R, et al. Expansive generation of functional airway epithelium from human embryonic stem cells. Stem Cells Transl Med. 2014;3(1):7–17.
   PubMed Web of Science ®Google Scholar
• Yoshie S, Imaizumi M, Nakamura R, et al. Generation of airway epithelial cells with native characteristics from mouse induced pluripotent stem cells. Cell Tissue Res. 2016;364(2):319–330.
   PubMed Web of Science ®Google Scholar
• Zhou Q, Ye X, Sun R, et al. Differentiation of mouse induced pluripotent stem cells into alveolar epithelial cells in vitro for use in vivo. Stem Cells Transl Med. 2014;3(6):675–685.
   PubMed Web of Science ®Google Scholar
• Hawkins F, Kotton DN. Embryonic and induced pluripotent stem cells for lung regeneration. Ann Am Thorac Soc. 2015;12(Suppl 1):S50–3.
   PubMedGoogle Scholar
• Wong AP, Rossant J. Generation of lung epithelium from pluripotent stem cells. Curr Pathobiol Rep. 2013;1(2):137–145.
   PubMedGoogle Scholar
• Wong AP, Bear CE, Chin S, et al. Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein. Nat Biotechnol. 2012;30(9):876–882.
   PubMed Web of Science ®Google Scholar
• Mou H, Zhao R, Sherwood R, et al. Generation of multipotent lung and airway progenitors from mouse ESCs and patient-specific cystic fibrosis iPSCs. Cell Stem Cell. 2012;10(4):385–397.
   PubMed Web of Science ®Google Scholar
• Firth AL, Menon T, Parker GS, et al. Functional gene correction for cystic fibrosis in lung epithelial cells generated from patient iPSCs. Cell Rep. 2015;12(9):1385–1390.
   PubMed Web of Science ®Google Scholar
• Yang KY, Shih HC, How CK, et al. IV delivery of induced pluripotent stem cells attenuates endotoxin-induced acute lung injury in mice. Chest. 2011;140(5):1243–1253.
   PubMed Web of Science ®Google Scholar
• How CK, Hou SK, Chen LK, et al. Induced pluripotent stem cells alleviate lung injury from mesenteric ischemia-reperfusion. J Trauma Acute Care Surg. 2015;79(4):592–601.
   PubMed Web of Science ®Google Scholar
• Su VY, Chiou SH, Lin CS, et al. Induced pluripotent stem cells reduce neutrophil chemotaxis via activating GRK2 in endotoxin-induced acute lung injury. Respirology. 2017;22(6):1156–1164.
   PubMed Web of Science ®Google Scholar
• Gui L, Qian H, Rocco KA, et al. Efficient intratracheal delivery of airway epithelial cells in mice and pigs. Am J Physiol Lung Cell Mol Physiol. 2015;308(2):L221–8.
   PubMed Web of Science ®Google Scholar
• Sampaziotis F, de Brito MC, Madrigal P, et al. Cholangiocytes derived from human induced pluripotent stem cells for disease modeling and drug validation. Nat Biotechnol. 2015;33(8):845–852.
   PubMed Web of Science ®Google Scholar
• Ogawa M, Ogawa S, Bear CE, et al. Directed differentiation of cholangiocytes from human pluripotent stem cells. Nat Biotechnol. 2015;33(8):853–861.
   PubMed Web of Science ®Google Scholar
• Simsek S, Zhou T, Robinson CL, et al. Modeling cystic fibrosis using pluripotent stem cell-derived human pancreatic ductal epithelial cells. Stem Cells Transl Med. 2016;5(5):572–579.
   PubMed Web of Science ®Google Scholar
• Sampaziotis F, Justin AW, Tysoe OC, et al. Reconstruction of the mouse extrahepatic biliary tree using primary human extrahepatic cholangiocyte organoids. Nat Med. 2017;23(8):954–963.
   PubMed Web of Science ®Google Scholar
• Krause DS, Theise ND, Collector MI, et al. Multi-organ, multi-lineage engraftment by a single bone marrow-derived stem cell. Cell. 2001;105(3):369–377.
   PubMed Web of Science ®Google Scholar
• Piro D, Rejman J, Conese M. Stem cell therapy for cystic fibrosis: current status and future prospects. Expert Rev Respir Med. 2008;2(3):365–380.
   PubMedGoogle Scholar
• McDonald LT, LaRue AC. Bone marrow stem cell contribution to pulmonary homeostasis and disease. J Bone Marrow Res. 2015;3(3).
   PubMedGoogle Scholar
• Piro D, Piccoli C, Guerra L, et al. Hematopoietic stem/progenitor cells express functional mitochondrial energy-dependent cystic fibrosis transmembrane conductance regulator. Stem Cells Dev. 2012;21(4):634–646.
   PubMed Web of Science ®Google Scholar
• Prockop DJ, Prockop SE, Bertoncello I. Are clinical trials with mesenchymal stem/progenitor cells too far ahead of the science? Lessons from experimental hematology. Stem Cells. 2014;32(12):3055–3061.
   PubMed Web of Science ®Google Scholar
• Sugimura R, Jha DK, Han A, et al. Haematopoietic stem and progenitor cells from human pluripotent stem cells. Nature. 2017;545(7655):432–438.
   PubMed Web of Science ®Google Scholar
• Lis R, Karrasch CC, Poulos MG, et al. Conversion of adult endothelium to immunocompetent haematopoietic stem cells. Nature. 2017;545(7655):439–445.
   PubMed Web of Science ®Google Scholar
• Yui S, Nakamura T, Sato T, et al. Functional engraftment of colon epithelium expanded in vitro from a single adult Lgr5(+) stem cell. Nat Med. 2012;18(4):618–623.
   PubMed Web of Science ®Google Scholar
• Dekkers JF, Wiegerinck CL, De Jonge HR, et al. A functional CFTR assay using primary cystic fibrosis intestinal organoids. Nat Med. 2013;19(7):939–945.
   PubMed Web of Science ®Google Scholar
• Schwank G, Koo BK, Sasselli V, et al. Functional repair of CFTR by CRISPR/Cas9 in intestinal stem cell organoids of cystic fibrosis patients. Cell Stem Cell. 2013;13(6):653–658.
   PubMed Web of Science ®Google Scholar
• Crisan M, Yap S, Casteilla L, et al. A perivascular origin for mesenchymal stem cells in multiple human organs. Cell Stem Cell. 2008;3(3):301–313.
   PubMed Web of Science ®Google Scholar
• Wagner W, Wein F, Seckinger A, et al. Comparative characteristics of mesenchymal stem cells from human bone marrow, adipose tissue, and umbilical cord blood. Exp Hematol. 2005;33(11):1402–1416.
   PubMed Web of Science ®Google Scholar
• Kern S, Eichler H, Stoeve J, et al. Comparative analysis of mesenchymal stem cells from bone marrow, umbilical cord blood, or adipose tissue. Stem Cells. 2006;24(5):1294–1301.
   PubMed Web of Science ®Google Scholar
• Al-Nbaheen M, Vishnubalaji R, Ali D, et al. Human stromal (mesenchymal) stem cells from bone marrow, adipose tissue and skin exhibit differences in molecular phenotype and differentiation potential. Stem Cell Rev. 2013;9(1):32–43.
   PubMed Web of Science ®Google Scholar
• Wang G, Bunnell BA, Painter RG, et al. Adult stem cells from bone marrow stroma differentiate into airway epithelial cells: potential therapy for cystic fibrosis. Proc Natl Acad Sci U S A. 2005;102(1):186–191.
   PubMed Web of Science ®Google Scholar
• Hayes M, Curley G, Ansari B, et al. Clinical review: stem cell therapies for acute lung injury/acute respiratory distress syndrome - hope or hype? Crit Care. 2012;16(2):205.
   PubMed Web of Science ®Google Scholar
• Zhu YG, Feng XM, Abbott J, et al. Human mesenchymal stem cell microvesicles for treatment of Escherichia coli endotoxin-induced acute lung injury in mice. Stem Cells. 2014;32(1):116–125.
   PubMed Web of Science ®Google Scholar
• Islam MN, Das SR, Emin MT, et al. Mitochondrial transfer from bone-marrow-derived stromal cells to pulmonary alveoli protects against acute lung injury. Nat Med. 2012;18(5):759–765.
   PubMed Web of Science ®Google Scholar
• Badri L, Walker NM, Ohtsuka T, et al. Epithelial interactions and local engraftment of lung-resident mesenchymal stem cells. Am J Respir Cell Mol Biol. 2011;45:809–816.
   PubMed Web of Science ®Google Scholar
• Conese M, Carbone A, Castellani S, et al. Paracrine effects and heterogeneity of marrow-derived stem/progenitor cells: relevance for the treatment of respiratory diseases. Cells Tissues Organs. 2013;197(6):445–473.
   PubMed Web of Science ®Google Scholar
• Inamdar AC, Inamdar AA. Mesenchymal stem cell therapy in lung disorders: pathogenesis of lung diseases and mechanism of action of mesenchymal stem cell. Exp Lung Res. 2013;39(8):315–327.
   PubMed Web of Science ®Google Scholar
• Geiger S, Hirsch D, Hermann FG. Cell therapy for lung disease. Eur Respir Rev. 2017;30;26:144.
   Web of Science ®Google Scholar
• Gu W, Song L, Li XM, et al. Mesenchymal stem cells alleviate airway inflammation and emphysema in COPD through down-regulation of cyclooxygenase-2 via p38 and ERK MAPK pathways. Sci Rep. 2015;5:8733.
   PubMed Web of Science ®Google Scholar
• Bonfield TL, Lennon D, Ghosh SK, et al. Cell based therapy aides in infection and inflammation resolution in the murine model of cystic fibrosis lung disease. Stem Cell Discov. 2013;3(2):139–153.
   Google Scholar
• Sutton MT, Fletcher D, Ghosh SK, et al. Antimicrobial properties of mesenchymal stem cells: therapeutic potential for cystic fibrosis infection, and treatment. Stem Cells Int. 2016;2016:5303048.
   PubMed Web of Science ®Google Scholar
• Bruscia EM, Price JE, Cheng E-C, et al. Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation. Proc Natl Acad Sci U S A. 2006;103:2965–2970.
   PubMed Web of Science ®Google Scholar
• Loi R, Beckett T, Goncz KK, et al. Limited restoration of cystic fibrosis lung epithelium in vivo with adult marrow derived cells. Am J Respir Crit Care Med. 2006;173:171–179.
   PubMed Web of Science ®Google Scholar
• Wong AP, Dutly AE, Sacher A, et al. Targeted cell replacement with bone marrow cells for airway epithelial regeneration. Am J Physiol Lung Cell Mol Physiol. 2007;293(3):L740–52.
   PubMed Web of Science ®Google Scholar
• Duchesneau P, Wong AP, Waddell TK. Optimization of targeted cell replacement therapy: a new approach for lung disease. Mol Ther. 2010;18(10):1830–1836.
   PubMed Web of Science ®Google Scholar
• Duchesneau P, Besla R, Derouet MF, et al. Partial restoration of CFTR function in cftr-null mice following targeted cell replacement therapy. Mol Ther. 2017;25(3):654–665.
   PubMed Web of Science ®Google Scholar
• Murphy SV, Atala A. Amniotic fluid and placental membranes: unexpected sources of highly multipotent cells. Semin Reprod Med. 2013;31(1):62–68.
   PubMed Web of Science ®Google Scholar
• Parolini O, Alviano F, Bergwerf I, et al. Toward cell therapy using placenta-derived cells: disease mechanisms, cell biology, preclinical studies, and regulatory aspects at the round table. Stem Cells Dev. 2010;19(2):143–154.
   PubMed Web of Science ®Google Scholar
• Silini A, Parolini O, Huppertz B, et al. Soluble factors of amnion-derived cells in treatment of inflammatory and fibrotic pathologies. Curr Stem Cell Res Ther. 2013;8(1):6–14.
   PubMed Web of Science ®Google Scholar
• Murphy S, Lim R, Dickinson H, et al. Human amnion epithelial cells prevent bleomycin-induced lung injury and preserve lung function. Cell Transplant. 2011;20(6):909–923.
   PubMed Web of Science ®Google Scholar
• Murphy SV, Shiyun SC, Tan JL, et al. Human amnion epithelial cells do not abrogate pulmonary fibrosis in mice with impaired macrophage function. Cell Transplant. 2012;21(7):1477–1492.
   PubMed Web of Science ®Google Scholar
• Murphy SV, Lim R, Heraud P, et al. Human amnion epithelial cells induced to express functional cystic fibrosis transmembrane conductance regulator. PLoS One. 2012;7(9):e46533.
   PubMed Web of Science ®Google Scholar
• Paracchini V, Carbone A, Colombo F, et al. Amniotic mesenchymal stem cells: a new source for hepatocyte-like cells and induction of CFTR expression by coculture with cystic fibrosis airway epithelial cells. J Biomed Biotechnol. 2012;2012:575471.
   PubMed Web of Science ®Google Scholar
• Carbone A, Castellani S, Favia M, et al. Correction of defective CFTR/ENaC function and tightness of cystic fibrosis airway epithelium by amniotic mesenchymal stromal (stem) cells. J Cell Mol Med. 2014;18(8):1631–1643.
   PubMed Web of Science ®Google Scholar
• Lavelle GM, White MM, Browne N, et al. Animal models of cystic fibrosis pathology: phenotypic parallels and divergences. Biomed Res Int. 2016;2016:5258727.
   PubMedGoogle Scholar
• Yan Z, Stewart ZA, Sinn PL, et al. Ferret and pig models of cystic fibrosis: prospects and promise for gene therapy. Hum Gene Ther Clin Dev. 2015;26(1):38–49.
   PubMed Web of Science ®Google Scholar
• Steines B, Dickey DD, Bergen J, et al. CFTR gene transfer with AAV improves early cystic fibrosis pig phenotypes. JCI Insight. 2016;1(14):e88728.
   PubMed Web of Science ®Google Scholar
• Cooney AL, Abou Alaiwa MH, Shah VS, et al. Lentiviral-mediated phenotypic correction of cystic fibrosis pigs. JCI Insight. 2016;1(14).
   PubMed Web of Science ®Google Scholar
• Forster R, Bode G, Ellegaard L, et al. The RETHINK project–minipigs as models for the toxicity testing of new medicines and chemicals: an impact assessment. J Pharmacol Toxicol Methods. 2010;62(3):158–159.
   PubMed Web of Science ®Google Scholar
• Dye BR, Hill DR, Ferguson MA, et al. In vitro generation of human pluripotent stem cell derived lung organoids. Elife. 2015;4:e05098.
   Web of Science ®Google Scholar
• Mondrinos MJ, Koutzaki S, Jiwanmall E, et al. Engineering three-dimensional pulmonary tissue constructs. Tissue Eng. 2006;12(4):717–728.
   PubMedGoogle Scholar
• Mondrinos MJ, Koutzaki S, Lelkes PI, et al. A tissue-engineered model of fetal distal lung tissue. Am J Physiol Lung Cell Mol Physiol. 2007;293(3):L639–50.
   PubMed Web of Science ®Google Scholar
• Cortiella J, Nichols JE, Kojima K, et al. Tissue-engineered lung: an in vivo and in vitro comparison of polyglycolic acid and pluronic F-127 hydrogel/somatic lung progenitor cell constructs to support tissue growth. Tissue Eng. 2006;12(5):1213–1225.
   PubMedGoogle Scholar
• Wagner DE, Bonvillain RW, Jensen T, et al. Can stem cells be used to generate new lungs? Ex vivo lung bioengineering with decellularized whole lung scaffolds. Respirology. 2013;18(6):895–911.
   PubMed Web of Science ®Google Scholar
• Cortiella J, Niles J, Cantu A, et al. Influence of acellular natural lung matrix on murine embryonic stem cell differentiation and tissue formation. Tissue Eng Part A. 2010;16(8):2565–2580.
   PubMed Web of Science ®Google Scholar
• Longmire TA, Ikonomou L, Hawkins F, et al. Efficient derivation of purified lung and thyroid progenitors from embryonic stem cells. Cell Stem Cell. 2012;10(4):398–411.
   PubMed Web of Science ®Google Scholar
• Shojaie S, Ermini L, Ackerley C, et al. Acellular lung scaffolds direct differentiation of endoderm to functional airway epithelial cells: requirement of matrix-bound HS proteoglycans. Stem Cell Reports. 2015;4(3):419–430.
   PubMed Web of Science ®Google Scholar
• Jensen T, Roszell B, Zang F, et al. A rapid lung de-cellularization protocol supports embryonic stem cell differentiation in vitro and following implantation. Tissue Eng Part C Methods. 2012;18(8):632–646.
   PubMed Web of Science ®Google Scholar
• Ghaedi M, Calle EA, Mendez JJ, et al. Human iPS cell-derived alveolar epithelium repopulates lung extracellular matrix. J Clin Invest. 2013;123(11):4950–4962.
   PubMed Web of Science ®Google Scholar
• Gilpin SE, Ren X, Okamoto T, et al. Enhanced lung epithelial specification of human induced pluripotent stem cells on decellularized lung matrix. Ann Thorac Surg. 2014;98(5):1721–1729.
   PubMed Web of Science ®Google Scholar
• Daly AB, Wallis JM, Borg ZD, et al. Initial binding and recellularization of decellularized mouse lung scaffolds with bone marrow-derived mesenchymal stromal cells. Tissue Eng Part A. 2012;18(1–2):1–16.
   PubMed Web of Science ®Google Scholar
• Wallis JM, Borg ZD, Daly AB, et al. Comparative assessment of detergent-based protocols for mouse lung de-cellularization and re-cellularization. Tissue Eng Part C Methods. 2012;18(6):420–432.
   PubMed Web of Science ®Google Scholar
• Bonvillain RW, Danchuk S, Sullivan DE, et al. A nonhuman primate model of lung regeneration: detergent-mediated decellularization and initial in vitro recellularization with mesenchymal stem cells. Tissue Eng Part A. 2012;18(23–24):2437–2452.
   PubMed Web of Science ®Google Scholar
• Bonenfant NR, Sokocevic D, Wagner DE, et al. The effects of storage and sterilization on de-cellularized and re-cellularized whole lung. Biomaterials. 2013;34(13):3231–3245.
   PubMed Web of Science ®Google Scholar
• Sokocevic D, Bonenfant NR, Wagner DE, et al. The effect of age and emphysematous and fibrotic injury on the re-cellularization of de-cellularized lungs. Biomaterials. 2013;34(13):3256–3269.
   PubMed Web of Science ®Google Scholar
• Mendez JJ, Ghaedi M, Steinbacher D, et al. Epithelial cell differentiation of human mesenchymal stromal cells in decellularized lung scaffolds. Tissue Eng Part A. 2014;20(11–12):1735–1746.
   PubMed Web of Science ®Google Scholar
• Crabbe A, Liu Y, Sarker SF, et al. Recellularization of decellularized lung scaffolds is enhanced by dynamic suspension culture. PLoS One. 2015;10(5):e0126846.
   PubMed Web of Science ®Google Scholar
• Wagner DE, Bonenfant NR, Parsons CS, et al. Comparative decellularization and recellularization of normal versus emphysematous human lungs. Biomaterials. 2014;35(10):3281–3297.
   PubMed Web of Science ®Google Scholar
• Jungebluth P, Bader A, Baiguera S, et al. The concept of in vivo airway tissue engineering. Biomaterials. 2012;33(17):4319–4326.
   PubMed Web of Science ®Google Scholar
• Wang LT, Ting CH, Yen ML, et al. Human mesenchymal stem cells (MSCs) for treatment towards immune- and inflammation-mediated diseases: review of current clinical trials. J Biomed Sci.2016;23(1):76.
   Google Scholar
• Chambers DC, Enever D, Ilic N, et al. A phase 1b study of placenta-derived mesenchymal stromal cells in patients with idiopathic pulmonary fibrosis. Respirology. 2014;19(7):1013–1018.
   PubMed Web of Science ®Google Scholar
• Walles T. Tracheobronchial bio-engineering: biotechnology fulfilling unmet medical needs. Adv Drug Deliv Rev. 2011;63(4–5):367–374.
   PubMed Web of Science ®Google Scholar
• Macchiarini P, Jungebluth P, Go T, et al. Clinical transplantation of a tissue-engineered airway. Lancet. 2008;372(9655):2023–2030.
   PubMed Web of Science ®Google Scholar
• Bader A, Macchiarini P. Moving towards in situ tracheal regeneration: the bionic tissue engineered transplantation approach. J Cell Mol Med. 2010;14(7):1877–1889.
   PubMed Web of Science ®Google Scholar
• Badylak SF, Weiss DJ, Caplan A, et al. Engineered whole organs and complex tissues. Lancet. 2012;379(9819):943–952.
   PubMed Web of Science ®Google Scholar
• Vogel G Another scathing report causes more eminent heads to roll in the Macchiarini scandal. Science news. 2016 [cited 2017 Nov 12]. Available from: http://www.sciencemag.org/news/2016/09/another-scathing-report-causes-more-eminent-heads-roll-macchiarini-scandal.
   Google Scholar
• Vogel G. 2017. Six papers by disgraced surgeon should be retracted, report concludes. Science news; [cited 2017 Nov 13]. Available from: http://www.sciencemag.org/news/2017/10/six-papers-disgraced-surgeon-should-be-retracted-report-concludes.
   Google Scholar