Gene Symbol: Sftpb
Description: surfactant associated protein B
Alias: AI562151, SF-B, SP-B, Sftp-3, Sftp3, pulmonary surfactant-associated protein B, pulmonary surfactant-associated proteolipid SPL(Phe)
Species: mouse
Products:     Sftpb

Top Publications

  1. Clark J, Wert S, Bachurski C, Stahlman M, Stripp B, Weaver T, et al. Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice. Proc Natl Acad Sci U S A. 1995;92:7794-8 pubmed
    ..Ablation of the SP-B gene disrupts the routing, storage, and function of surfactant phospholipids and proteins, causing respiratory failure at birth. ..
  2. Bridges J, Ikegami M, Brilli L, Chen X, Mason R, Shannon J. LPCAT1 regulates surfactant phospholipid synthesis and is required for transitioning to air breathing in mice. J Clin Invest. 2010;120:1736-48 pubmed publisher
    ..Collectively, these data demonstrate that full LPCAT1 activity is required to achieve the levels of SatPC essential for the transition to air breathing. ..
  3. Cole T, Blendy J, Monaghan A, Krieglstein K, Schmid W, Aguzzi A, et al. Targeted disruption of the glucocorticoid receptor gene blocks adrenergic chromaffin cell development and severely retards lung maturation. Genes Dev. 1995;9:1608-21 pubmed
  4. Melton K, Nesslein L, Ikegami M, Tichelaar J, Clark J, Whitsett J, et al. SP-B deficiency causes respiratory failure in adult mice. Am J Physiol Lung Cell Mol Physiol. 2003;285:L543-9 pubmed
    ..Reduction of alveolar SP-B content causes surfactant dysfunction and respiratory failure, indicating that SP-B is required for postnatal lung function. ..
  5. Mucenski M, Wert S, Nation J, Loudy D, Huelsken J, Birchmeier W, et al. beta-Catenin is required for specification of proximal/distal cell fate during lung morphogenesis. J Biol Chem. 2003;278:40231-8 pubmed
    ..These observations establish, for the first time, both the sites and timing of specification of the proximal and peripheral airways in the developing lung, and that beta-catenin is one of the essential components of this specification. ..
  6. Cao Y, Vo T, Millien G, Tagne J, Kotton D, Mason R, et al. Epigenetic mechanisms modulate thyroid transcription factor 1-mediated transcription of the surfactant protein B gene. J Biol Chem. 2010;285:2152-64 pubmed publisher
    ..Surfactant protein B (Sftpb) is a developmentally regulated lung epithelial gene critical for lung function...
  7. Geng Y, Dong Y, Yu M, Zhang L, Yan X, Sun J, et al. Follistatin-like 1 (Fstl1) is a bone morphogenetic protein (BMP) 4 signaling antagonist in controlling mouse lung development. Proc Natl Acad Sci U S A. 2011;108:7058-63 pubmed publisher
    ..Therefore, we provide in vivo and in vitro evidence to demonstrate that Fstl1 modulates lung development and alveolar maturation, in part, through BMP4 signaling...
  8. D Amore Bruno M, Wikenheiser K, Carter J, Clark J, Whitsett J. Sequence, ontogeny, and cellular localization of murine surfactant protein B mRNA. Am J Physiol. 1992;262:L40-7 pubmed
    ..The murine SP-B mRNA was expressed in proximal and distal epithelial cells within the respiratory tract and shares a close structural relationship to SP-B from other species...
  9. Bellusci S, Furuta Y, Rush M, Henderson R, Winnier G, Hogan B. Involvement of Sonic hedgehog (Shh) in mouse embryonic lung growth and morphogenesis. Development. 1997;124:53-63 pubmed
    ..By contrast, Ptc is clearly upregulated in the transgenic lung. These results thus establish a role for SHH in lung morphogenesis, and suggest that SHH normally regulates lung mesenchymal cell proliferation in vivo. ..

More Information


  1. Yang L, Naltner A, Kreiner A, Yan D, Cowen A, Du H, et al. An enhancer region determines hSP-B gene expression in bronchiolar and ATII epithelial cells in transgenic mice. Am J Physiol Lung Cell Mol Physiol. 2003;284:L481-8 pubmed
    ..5-kb lacZ gene. The deletion abolished hSP-B lacZ gene expression in bronchiolar epithelial cells and significantly reduced its expression level in alveolar type II epithelial cells in transgenic mice. ..
  2. Li C, Xiao J, Hormi K, Borok Z, Minoo P. Wnt5a participates in distal lung morphogenesis. Dev Biol. 2002;248:68-81 pubmed
    ..Absence of WNT5a activity in the mutant lungs leads to increased expression of Fgf-10, Bmp4, Shh, and its receptor Ptc, raising the possibility that WNT5a, FGF-10, BMP4, and SHH signaling pathways are functionally interactive. ..
  3. Okubo T, Knoepfler P, Eisenman R, Hogan B. Nmyc plays an essential role during lung development as a dosage-sensitive regulator of progenitor cell proliferation and differentiation. Development. 2005;132:1363-74 pubmed
    ..We propose a model in which Nmyc is essential in the developing lung for maintaining a distal population of undifferentiated, proliferating progenitor cells. ..
  4. O Brien K, Alberich Jorda M, Yadav N, Kocher O, Diruscio A, Ebralidze A, et al. CARM1 is required for proper control of proliferation and differentiation of pulmonary epithelial cells. Development. 2010;137:2147-56 pubmed publisher
    ..These results demonstrate for the first time that CARM1 inhibits pulmonary cell proliferation and is required for proper differentiation of alveolar cells. ..
  5. Bird A, Flecknoe S, Tan K, Olsson P, Antony N, Mantamadiotis T, et al. cAMP response element binding protein is required for differentiation of respiratory epithelium during murine development. PLoS ONE. 2011;6:e17843 pubmed publisher
    ..Together, these results demonstrate a crucial role for Creb1 activity for the development and differentiation of the conducting and distal lung epithelium. ..
  6. Grad I, McKee T, Ludwig S, Hoyle G, Ruiz P, Wurst W, et al. The Hsp90 cochaperone p23 is essential for perinatal survival. Mol Cell Biol. 2006;26:8976-83 pubmed
    ..Thus, p23 contributes a nonredundant, temporally restricted, and tissue-specific function during mouse development. ..
  7. Okubo T, Hogan B. Hyperactive Wnt signaling changes the developmental potential of embryonic lung endoderm. J Biol. 2004;3:11 pubmed
    ..We discuss the relevance of our findings to the poorly understood pathological condition of intestinal metaplasia in humans. ..
  8. DeFelice M, Silberschmidt D, DiLauro R, Xu Y, Wert S, Weaver T, et al. TTF-1 phosphorylation is required for peripheral lung morphogenesis, perinatal survival, and tissue-specific gene expression. J Biol Chem. 2003;278:35574-83 pubmed
    ..Direct and indirect transcriptional targets of TTF-1 were identified that are likely to play important roles in lung formation and function. ..
  9. Wan H, Xu Y, Ikegami M, Stahlman M, Kaestner K, Ang S, et al. Foxa2 is required for transition to air breathing at birth. Proc Natl Acad Sci U S A. 2004;101:14449-54 pubmed
    ..Foxa2 regulates a complex pulmonary program of epithelial cell maturation required for transition to air breathing at birth. ..
  10. Zhou L, Lim L, Costa R, Whitsett J. Thyroid transcription factor-1, hepatocyte nuclear factor-3beta, surfactant protein B, C, and Clara cell secretory protein in developing mouse lung. J Histochem Cytochem. 1996;44:1183-93 pubmed
    ..The early expression of TTF-1 and HNF-3beta, preceding and overlapping that of pro-SP-B, mature SP-B, pro-SP-C, and CCSP, supports a regulatory role for TTF-1 and HNF-3beta in lung-specific gene expression. ..
  11. Epaud R, Ikegami M, Whitsett J, Jobe A, Weaver T, Akinbi H. Surfactant protein B inhibits endotoxin-induced lung inflammation. Am J Respir Cell Mol Biol. 2003;28:373-8 pubmed
  12. Field Corbett C, English K, O Dea S. Regulation of surfactant protein B gene expression in bone marrow-derived cells. Stem Cells. 2009;27:662-9 pubmed publisher
    ..Furthermore, silencing events, such as methylation, may occur to suppress lung-related gene expression as progenitor cells become committed toward more mature hematopoietic cell phenotypes. ..
  13. Xu Y, Wang Y, Besnard V, Ikegami M, Wert S, Heffner C, et al. Transcriptional programs controlling perinatal lung maturation. PLoS ONE. 2012;7:e37046 pubmed publisher
    ..The present study systemically mapped key regulators, bioprocesses, and transcriptional networks controlling lung maturation, providing the basis for new therapeutic strategies to enhance lung function in preterm infants. ..
  14. Nasonkin I, Ward R, Raetzman L, Seasholtz A, Saunders T, Gillespie P, et al. Pituitary hypoplasia and respiratory distress syndrome in Prop1 knockout mice. Hum Mol Genet. 2004;13:2727-35 pubmed
    ..Prop1-null mice are an excellent model for MPHD and may be useful for testing the efficacy of pharmaceutical intervention for neonatal respiratory distress. ..
  15. Bridges J, Ludwig M, Mueller M, Kinzel B, Sato A, Xu Y, et al. Orphan G protein-coupled receptor GPR116 regulates pulmonary surfactant pool size. Am J Respir Cell Mol Biol. 2013;49:348-57 pubmed publisher
    ..Collectively, these data support the concept that GPR116 functions as a molecular sensor of alveolar surfactant lipid pool sizes by regulating surfactant secretion. ..
  16. Tremblay L, Nagy Kovács E, Daniels E, Wong N, Sutton Smith M, Morris H, et al. Respiratory distress and neonatal lethality in mice lacking Golgi alpha1,2-mannosidase IB involved in N-glycan maturation. J Biol Chem. 2007;282:2558-66 pubmed
    ..The alpha1,2-mannosidase IB null phenotype differs from phenotypes caused by ablation of other enzymes in N-glycan biosynthesis and from other mouse gene disruptions that affect pulmonary development and function. ..
  17. Moreno Barriuso N, López Malpartida A, de Pablo F, Pichel J. Alterations in alveolar epithelium differentiation and vasculogenesis in lungs of LIF/IGF-I double deficient embryos. Dev Dyn. 2006;235:2040-50 pubmed
    ..The differentiation of type I cells barely occurred and capillaries remained in the abundant mesenchyme. These results indicate that LIF collaborates with IGF-I in lung alveolar epithelium and vascular maturation. ..
  18. Prows D, McDowell S, Aronow B, Leikauf G. Genetic susceptibility to nickel-induced acute lung injury. Chemosphere. 2003;51:1139-48 pubmed
    ..Together, results from QTL and microarray analyses of nickel-induced acute lung injury survival allowed us to generate a short list of candidate genes. ..
  19. Schwingshackl A, Lopez B, Teng B, Luellen C, Lesage F, Belperio J, et al. Hyperoxia treatment of TREK-1/TREK-2/TRAAK-deficient mice is associated with a reduction in surfactant proteins. Am J Physiol Lung Cell Mol Physiol. 2017;313:L1030-L1046 pubmed publisher
    ..1/thyroid transcription factor-1 (TTF-1) or c-jun, or lamellar body levels. Prophylactic Curosurf administration did not improve lung injury scores or compliance in triple KO mice. ..
  20. Ikegami M, Takabatake N, Weaver T. Intersubunit disulfide bridge is not required for the protective role of SP-B against lung inflammation. J Appl Physiol (1985). 2002;93:505-11 pubmed
    ..Overall, increased inflammation in SP-Bmon(+) mice was corrected to a large extent by increased gene dosage, indicating that formation of the intersubunit disulfide bridge is not critical for SP-B function. ..
  21. Ringvall M, Ledin J, Holmborn K, van Kuppevelt T, Ellin F, Eriksson I, et al. Defective heparan sulfate biosynthesis and neonatal lethality in mice lacking N-deacetylase/N-sulfotransferase-1. J Biol Chem. 2000;275:25926-30 pubmed
    ..In addition, a minor proportion of NDST-1-deficient embryos die during the embryonic period. The cause of the embryonic lethality is still obscure, but incompletely penetrant defects of the skull and the eyes have been observed. ..
  22. Zhang J, Liu Z, Zhang T, Lin Z, Li Z, Zhang A, et al. Loss of Lysyl Oxidase-like 3 Attenuates Embryonic Lung Development in Mice. Sci Rep. 2016;6:33856 pubmed publisher
    ..Increased transforming growth factor ?1 (TGF?1) expression might be involved in the up-regulation of LOXL4 in LOXL3-deficient lungs. We concluded that the loss of LOXL3 attenuates mouse embryonic lung development. ..
  23. Stahlman M, Gray M, Falconieri M, Whitsett J, Weaver T. Lamellar body formation in normal and surfactant protein B-deficient fetal mice. Lab Invest. 2000;80:395-403 pubmed
    ..Taken together, these observations suggest that vesicular inclusions in SP-B -/- mice are disorganized lamellar bodies in which the absence of SP-B leads to failure to package surfactant phospholipids into concentric lamellae. ..
  24. Korfhagen T, Levine A, Whitsett J. Surfactant protein A (SP-A) gene targeted mice. Biochim Biophys Acta. 1998;1408:296-302 pubmed
    ..These studies of SP-A (-/-) mice demonstrate that SP-A has an important role in the innate immune system of the lung in vivo. ..
  25. Weaver T, Beck D. Use of knockout mice to study surfactant protein structure and function. Biol Neonate. 1999;76 Suppl 1:15-8 pubmed
    ..The effect of transgene expression on lung structure and function was assessed by biochemical, morphological and physiological analyses of the surfactant system in fetal and postnatal offspring. ..
  26. Shu W, Lu M, Zhang Y, Tucker P, Zhou D, Morrisey E. Foxp2 and Foxp1 cooperatively regulate lung and esophagus development. Development. 2007;134:1991-2000 pubmed
  27. Albert M, Schmitz S, Kooistra S, Malatesta M, Morales Torres C, Rekling J, et al. The histone demethylase Jarid1b ensures faithful mouse development by protecting developmental genes from aberrant H3K4me3. PLoS Genet. 2013;9:e1003461 pubmed publisher
    ..Taken together, these results suggest that Jarid1b regulates mouse development by protecting developmental genes from inappropriate acquisition of active histone modifications. ..
  28. Sun L, Lee D, Zhang Q, Xiao W, Raabe E, Meeker A, et al. Growth retardation and premature aging phenotypes in mice with disruption of the SNF2-like gene, PASG. Genes Dev. 2004;18:1035-46 pubmed
    ..PASG mutant mice provide a useful model for the study of aging as well as the mechanisms regulating epigenetic patterning during development and postnatal life...
  29. Li H, Witte D, Branford W, Aronow B, Weinstein M, Kaur S, et al. Gsh-4 encodes a LIM-type homeodomain, is expressed in the developing central nervous system and is required for early postnatal survival. EMBO J. 1994;13:2876-85 pubmed
    ..It is concluded that Gsh-4 plays a critical role in the development of respiratory control mechanisms and in the normal growth and maturation of the lung. ..
  30. Yang L, Johansson J, Ridsdale R, Willander H, Fitzen M, Akinbi H, et al. Surfactant protein B propeptide contains a saposin-like protein domain with antimicrobial activity at low pH. J Immunol. 2010;184:975-83 pubmed publisher
    ..These findings support the hypothesis that SP-BN contributes to innate host defense of the lung by supplementing the nonoxidant antimicrobial defenses of alveolar macrophages. ..
  31. Whiteman E, Fan S, Harder J, Walton K, Liu C, Soofi A, et al. Crumbs3 is essential for proper epithelial development and viability. Mol Cell Biol. 2014;34:43-56 pubmed publisher
    ..Taken together, our data indicate that Crumbs3 is crucial for epithelial morphogenesis and plays a role in linking the apical membrane to the underlying ezrin-containing cytoskeleton. ..
  32. Bruno M, Bohinski R, Carter J, Foss K, Whitsett J. Structure and function of the mouse surfactant protein B gene. Am J Physiol. 1995;268:L381-9 pubmed
    ..The 5' flanking region of the murine SP-B contains cell-specific cis-active elements that regulate SP-B expression in the respiratory epithelium...
  33. Ikegami M, Weaver T, Grant S, Whitsett J. Pulmonary surfactant surface tension influences alveolar capillary shape and oxygenation. Am J Respir Cell Mol Biol. 2009;41:433-9 pubmed publisher
    ..our hypothesis, surfactant surface tension was regulated by conditional expression of surfactant protein (SP)-B in Sftpb(-/-) mice, thereby inhibiting surface tension-lowering properties of surfactant in vivo within 24 hours after ..
  34. Akei H, Whitsett J, Buroker M, Ninomiya T, Tatsumi H, Weaver T, et al. Surface tension influences cell shape and phagocytosis in alveolar macrophages. Am J Physiol Lung Cell Mol Physiol. 2006;291:L572-9 pubmed
    ..Surface tension was regulated in vivo by conditionally expressing surfactant protein (SP)-B in Sftpb-/- mice...
  35. Dalvin S, Anselmo M, Prodhan P, Komatsuzaki K, Schnitzer J, Kinane T. Expression of Netrin-1 and its two receptors DCC and UNC5H2 in the developing mouse lung. Gene Expr Patterns. 2003;3:279-83 pubmed
    ..We also demonstrate localization of Netrin-1, DCC and UNC5H2 during the stages of lung development. We present evidence that these proteins are modulated spatially in the mesenchyme and epithelium during lung organogenesis. ..
  36. Fitzgerald M, Xavier R, Haley K, Welti R, Goss J, Brown C, et al. ABCA3 inactivation in mice causes respiratory failure, loss of pulmonary surfactant, and depletion of lung phosphatidylglycerol. J Lipid Res. 2007;48:621-32 pubmed
    ..They also demonstrate the utility of the Abca3 null mouse as a model for a devastating human disease...
  37. El Hashash A, Al Alam D, Turcatel G, Bellusci S, Warburton D. Eyes absent 1 (Eya1) is a critical coordinator of epithelial, mesenchymal and vascular morphogenesis in the mammalian lung. Dev Biol. 2011;350:112-26 pubmed publisher
    ..We conclude, therefore, that Eya1 function is critical for proper coordination of lung epithelial, mesenchymal and vascular development. ..
  38. Im S, Jeong H, Jeong H, Kim K, Hwang D, Ikegami M, et al. Disruption of sorting nexin 5 causes respiratory failure associated with undifferentiated alveolar epithelial type I cells in mice. PLoS ONE. 2013;8:e58511 pubmed publisher
    ..These results demonstrate that Snx5 is necessary for the differentiation of alveolar epithelial type I cells, which may underlie the adaptation to air breathing at birth...
  39. Leikauf G, McDowell S, Wesselkamper S, Miller C, Hardie W, Gammon K, et al. Pathogenomic mechanisms for particulate matter induction of acute lung injury and inflammation in mice. Res Rep Health Eff Inst. 2001;:5-58; discussion 59-71 pubmed
    ..Future assessment of these susceptibility genes (including evaluations of human synteny and function) could provide valuable insights into individual susceptibility to the adverse effects of particulate matter. ..
  40. Wan H, Dingle S, Xu Y, Besnard V, Kaestner K, Ang S, et al. Compensatory roles of Foxa1 and Foxa2 during lung morphogenesis. J Biol Chem. 2005;280:13809-16 pubmed
    ..Foxa family members regulate signaling and transcriptional programs required for morphogenesis and cell differentiation during formation of the lung. ..
  41. El Hashash A, Al Alam D, Turcatel G, Rogers O, Li X, Bellusci S, et al. Six1 transcription factor is critical for coordination of epithelial, mesenchymal and vascular morphogenesis in the mammalian lung. Dev Biol. 2011;353:242-58 pubmed publisher
    ..We propose that Six1 is hence critical for coordination of proper lung epithelial, mesenchymal and vascular development. ..
  42. Mohamed B, Barakat A, Held T, Elkenani M, Mühlfeld C, Männer J, et al. Respiratory distress and early neonatal lethality in Hspa4l/Hspa4 double-mutant mice. Am J Respir Cell Mol Biol. 2014;50:817-24 pubmed publisher
    ..Our study demonstrates that HSPA4L and HSPA4 collaborate in embryonic lung maturation, which is necessary for adaptation to air breathing at birth. ..
  43. Zhang Y, Goss A, Cohen E, Kadzik R, Lepore J, Muthukumaraswamy K, et al. A Gata6-Wnt pathway required for epithelial stem cell development and airway regeneration. Nat Genet. 2008;40:862-70 pubmed publisher
    ..Together, these data demonstrate that Gata6-regulated Wnt signaling controls the balance between progenitor expansion and epithelial differentiation required for both lung development and regeneration. ..
  44. Goshu E, Jin H, Fasnacht R, Sepenski M, Michaud J, Fan C. Sim2 mutants have developmental defects not overlapping with those of Sim1 mutants. Mol Cell Biol. 2002;22:4147-57 pubmed
    ..The temporal and spatial expression patterns of Sim2 in these skeletal elements suggest that Sim2 regulates their growth and/or integrity. ..
  45. Han R, Babaei S, Robb M, Lee T, Ridsdale R, Ackerley C, et al. Defective lung vascular development and fatal respiratory distress in endothelial NO synthase-deficient mice: a model of alveolar capillary dysplasia?. Circ Res. 2004;94:1115-23 pubmed
    ..We conclude that eNOS plays a previously unrecognized role in lung development, which may have relevance for clinical syndromes of neonatal respiratory distress. ..
  46. Fernandez Valdivia R, Zhang Y, Pai S, Metzker M, Schumacher A. l7Rn6 encodes a novel protein required for clara cell function in mouse lung development. Genetics. 2006;172:389-99 pubmed
    ..Thus, l7Rn6 represents a novel protein required for organization and/or function of the secretory apparatus in Clara cells in mouse lung. ..
  47. Lee S, Kim D, Kang J, Kim E, Kim W, Youn H, et al. Surfactant Protein B Suppresses Lung Cancer Progression by Inhibiting Secretory Phospholipase A2 Activity and Arachidonic Acid Production. Cell Physiol Biochem. 2017;42:1684-1700 pubmed publisher
    ..To investigate the role of SP-B in radioresistance, ?SFTPB A549 cell line was established and SP-B expression was analyzed...
  48. Fredenburgh L, Baron R, Carvajal I, Mouded M, Macias A, Ith B, et al. Absence of heme oxygenase-1 expression in the lung parenchyma exacerbates endotoxin-induced acute lung injury and decreases surfactant protein-B levels. Cell Mol Biol (Noisy-le-grand). 2005;51:513-20 pubmed
    ..These findings have implications for our understanding of the pathophysiology of ARDS and may guide future therapeutic strategies. ..
  49. Jia W, Jiang S, Tang Q, Shen D, Xue B, Ning W, et al. Geranylgeranyl Diphosphate Synthase Modulates Fetal Lung Branching Morphogenesis Possibly through Controlling K-Ras Prenylation. Am J Pathol. 2016;186:1454-65 pubmed publisher
    ..Collectively, our data suggest that GGPPS is essential for maintaining fetal lung branching morphogenesis, which is possibly through regulating K-Ras prenylation. ..
  50. Woik N, Dietz C, Schäker K, Kroll J. Kelch-like ECT2-interacting protein KLEIP regulates late-stage pulmonary maturation via Hif-2? in mice. Dis Model Mech. 2014;7:683-92 pubmed publisher
  51. Chen L, Wilson R, Bennett E, Zosky G. Identification of vitamin D sensitive pathways during lung development. Respir Res. 2016;17:47 pubmed publisher
    ..These data provided a plausible mechanism linking maternal vitamin D deficiency with altered postnatal lung function. ..
  52. Tong Q, Zheng L, Dodd O J, Langer J, Wang D, Li D. Hypoxia-induced mitogenic factor modulates surfactant protein B and C expression in mouse lung. Am J Respir Cell Mol Biol. 2006;34:28-38 pubmed
  53. Tokieda K, Iwamoto H, Bachurski C, Wert S, Hull W, Ikeda K, et al. Surfactant protein-B-deficient mice are susceptible to hyperoxic lung injury. Am J Respir Cell Mol Biol. 1999;21:463-72 pubmed
    ..These data support the concept that SP-B plays an important protective role in the lung. ..
  54. Li A, Hardy R, Stoner S, Tuckermann J, Seibel M, Zhou H. Deletion of mesenchymal glucocorticoid receptor attenuates embryonic lung development and abdominal wall closure. PLoS ONE. 2013;8:e63578 pubmed publisher
    ..In addition, altered glucocorticoid signaling in the mesenchyme attenuates normal lung epithelial differentiation...
  55. Xing Y, Li A, Borok Z, Li C, Minoo P. NOTCH1 is required for regeneration of Clara cells during repair of airway injury. Stem Cells. 2012;30:946-55 pubmed publisher
    ..The signaling pathway by which Notch1 regulates the repair process includes stimulation of Hes5 and Pax6 gene expression...
  56. Pryhuber G, Bachurski C, Hirsch R, Bacon A, Whitsett J. Tumor necrosis factor-alpha decreases surfactant protein B mRNA in murine lung. Am J Physiol. 1996;270:L714-21 pubmed
    ..TNF-alpha-mediated decrease in SP gene expression may contribute to the surfactant dysfunction and atelectasis observed in inflammatory lung diseases...
  57. Xu B, Chen C, Chen H, Zheng S, Bringas P, Xu M, et al. Smad1 and its target gene Wif1 coordinate BMP and Wnt signaling activities to regulate fetal lung development. Development. 2011;138:925-35 pubmed publisher
    ..This suggests a novel regulatory loop of Bmp4-Smad1-Wif1-Wnt/?-catenin in coordinating BMP and Wnt pathways to control fetal lung development. ..
  58. Yu W, Li X, Eliason S, Romero Bustillos M, Ries R, Cao H, et al. Irx1 regulates dental outer enamel epithelial and lung alveolar type II epithelial differentiation. Dev Biol. 2017;429:44-55 pubmed publisher
    ..Mechanistically, Irx1 regulates Foxj1 and Sox9 to control cell differentiation during development. ..
  59. Cardoso W, Itoh A, Nogawa H, Mason I, Brody J. FGF-1 and FGF-7 induce distinct patterns of growth and differentiation in embryonic lung epithelium. Dev Dyn. 1997;208:398-405 pubmed
    ..Thus, in the embryonic lung epithelium, growth effects of FGFs appear to be dependent on location of FGFRs, while effects on differentiation are ligand-dependent. ..
  60. Silva D, Venihaki M, Guo W, Lopez M. Igf2 deficiency results in delayed lung development at the end of gestation. Endocrinology. 2006;147:5584-91 pubmed
    ..This study suggests that Igf2 plays an important role in the development of the fetal lung and may affect fetal lung maturation by regulating maternal factors, such as corticosterone levels, during pregnancy. ..
  61. Conkright J, Na C, Weaver T. Overexpression of surfactant protein-C mature peptide causes neonatal lethality in transgenic mice. Am J Respir Cell Mol Biol. 2002;26:85-90 pubmed
    ..These results suggest that elevated expression of SP-C(24-57)HA in type II cells resulted in aggregation of SP-C in the early secretory pathway, leading to cytotoxicity and, ultimately, altered lung development. ..
  62. Zhang Y, Rath N, Hannenhalli S, Wang Z, Cappola T, Kimura S, et al. GATA and Nkx factors synergistically regulate tissue-specific gene expression and development in vivo. Development. 2007;134:189-98 pubmed
  63. Lee K, Lee Y, Lee J, Ito K, Cinghu S, Kim J, et al. Runx3 is required for the differentiation of lung epithelial cells and suppression of lung cancer. Oncogene. 2010;29:3349-61 pubmed publisher
    ..We conclude that Runx3 is essential for lung epithelial cell differentiation, and that downregulation of Runx3 is causally linked to the preneoplastic stage of lung adenocarcinoma. ..
  64. Zhang M, Liu X, Zhang Y, Zhao J. Loss of betaarrestin1 and betaarrestin2 contributes to pulmonary hypoplasia and neonatal lethality in mice. Dev Biol. 2010;339:407-17 pubmed publisher
    ..Together, our study demonstrates that betaarrestin1 and betaarrestin2 collaborate in embryonic development processes for epithelial pneumocyte differentiation and lung maturation. ..
  65. Xu B, Qu X, Gu S, Doughman Y, Watanabe M, Dunwoodie S, et al. Cited2 is required for fetal lung maturation. Dev Biol. 2008;317:95-105 pubmed publisher
    ..We propose that the Cited2-Tcfap2c complex controls lung maturation by regulating Cebpa expression. Understanding the function of this complex may provide novel therapeutic strategies for patients with respiratory distress syndromes. ..
  66. Zhang P, Wong C, Liu D, Finegold M, Harper J, Elledge S. p21(CIP1) and p57(KIP2) control muscle differentiation at the myogenin step. Genes Dev. 1999;13:213-24 pubmed
  67. Bachurski C, Pryhuber G, Glasser S, Kelly S, Whitsett J. Tumor necrosis factor-alpha inhibits surfactant protein C gene transcription. J Biol Chem. 1995;270:19402-7 pubmed
    ..Inhibition of surfactant protein C gene transcription by TNF-alpha may contribute to the abnormalities of surfactant homeostasis associated with pulmonary injury and infection. ..
  68. Otsubo K, Goto H, Nishio M, Kawamura K, Yanagi S, Nishie W, et al. MOB1-YAP1/TAZ-NKX2.1 axis controls bronchioalveolar cell differentiation, adhesion and tumour formation. Oncogene. 2017;36:4201-4211 pubmed publisher
    ..Thus, a MOB1-YAP1/TAZ-NKX2.1 axis is essential for normal lung homeostasis and expression of the collagen XVII protein necessary for alveolar stem cell maintenance in the lung niche. ..
  69. Ramasamy S, Mailleux A, Gupte V, Mata F, Sala F, Veltmaat J, et al. Fgf10 dosage is critical for the amplification of epithelial cell progenitors and for the formation of multiple mesenchymal lineages during lung development. Dev Biol. 2007;307:237-47 pubmed
    ..Thus, our results indicate that FGF10 plays a pivotal role in maintaining epithelial progenitor cell proliferation as well as coordinating alveolar smooth muscle cell formation and vascular development. ..
  70. Bader B, Smyth N, Nedbal S, Miosge N, Baranowsky A, Mokkapati S, et al. Compound genetic ablation of nidogen 1 and 2 causes basement membrane defects and perinatal lethality in mice. Mol Cell Biol. 2005;25:6846-56 pubmed
    ..However, despite the ubiquitous presence of nidogens in basement membranes, defects do not occur in all tissues or in all basement membranes, suggesting a varying spectrum of roles for nidogens in the basement membrane. ..
  71. Yu H, Wessels A, Chen J, Phelps A, Oatis J, Tint G, et al. Late gestational lung hypoplasia in a mouse model of the Smith-Lemli-Opitz syndrome. BMC Dev Biol. 2004;4:1 pubmed
    ..The molecular mechanism of impaired lung development associated with sterol deficiency by Dhcr7 loss is still unknown, but these results do not support the involvement of dysregulated Shh-Patched-Gli pathway in causing this defect. ..
  72. Davis R, Shen W, Sandler Y, Amoui M, Purcell P, Maas R, et al. Dach1 mutant mice bear no gross abnormalities in eye, limb, and brain development and exhibit postnatal lethality. Mol Cell Biol. 2001;21:1484-90 pubmed
    ..Since Dach1 mutants die shortly after birth, it remains possible that Dach1 is required for postnatal development of these structures. Alternatively, an additional Dach homologue may functionally compensate for Dach1 loss of function. ..
  73. Ridsdale R, Post M. Surfactant lipid synthesis and lamellar body formation in glycogen-laden type II cells. Am J Physiol Lung Cell Mol Physiol. 2004;287:L743-51 pubmed
  74. Muglia L, Bae D, Brown T, Vogt S, Alvarez J, Sunday M, et al. Proliferation and differentiation defects during lung development in corticotropin-releasing hormone-deficient mice. Am J Respir Cell Mol Biol. 1999;20:181-8 pubmed
  75. Sayin V, Nilton A, Ibrahim M, Agren P, Larsson E, Petit M, et al. Zfp148 deficiency causes lung maturation defects and lethality in newborn mice that are rescued by deletion of p53 or antioxidant treatment. PLoS ONE. 2013;8:e55720 pubmed publisher
    ..Our results establish Zfp148 as a novel player in mammalian lung maturation and demonstrate that Zfp148 is critical for cell cycle progression in vivo. ..
  76. Kishimoto K, Nishizuka M, Ueda T, Kajita K, Ugawa S, Shimada S, et al. Indispensable role of factor for adipocyte differentiation 104 (fad104) in lung maturation. Exp Cell Res. 2011;317:2110-23 pubmed publisher
    ..Collectively, these results indicate that fad104 has an indispensable role in lung maturation, especially the maturation and differentiation of ATII cells. ..
  77. Snitow M, Li S, Morley M, Rathi K, Lu M, Kadzik R, et al. Ezh2 represses the basal cell lineage during lung endoderm development. Development. 2015;142:108-17 pubmed publisher
    ..Together, these findings show that Ezh2 restricts the basal cell lineage during normal lung endoderm development to allow the proper patterning of epithelial lineages during lung formation. ..
  78. Mimura N, Hamada H, Kashio M, Jin H, Toyama Y, Kimura K, et al. Aberrant quality control in the endoplasmic reticulum impairs the biosynthesis of pulmonary surfactant in mice expressing mutant BiP. Cell Death Differ. 2007;14:1475-85 pubmed
    ..Moreover, failure of this adaptive mechanism may increase pulmonary susceptibility to environmental insults, such as hypoxia and ischemia, ultimately leading to neonatal respiratory failure. ..
  79. Liu W, Purevdorj E, Zscheppang K, von Mayersbach D, Behrens J, Brinkhaus M, et al. ErbB4 regulates the timely progression of late fetal lung development. Biochim Biophys Acta. 2010;1803:832-9 pubmed publisher
    ..We conclude that pulmonary ErbB4 deletion results in a structural and functional delay in fetal lung development, indicating a crucial regulatory role of ErbB4 in the timely progression of fetal lung development. ..
  80. Haczku A, Atochina E, Tomer Y, Cao Y, Campbell C, Scanlon S, et al. The late asthmatic response is linked with increased surface tension and reduced surfactant protein B in mice. Am J Physiol Lung Cell Mol Physiol. 2002;283:L755-65 pubmed
    ..Thus surfactant dysfunction induced by an IL-4-driven SP-B deficiency after allergen provocation may be an important part of the late asthmatic airway response. ..
  81. Lawson P, Reid K. The roles of surfactant proteins A and D in innate immunity. Immunol Rev. 2000;173:66-78 pubmed
    ..Linkage and polymorphism analysis is explaining the role these genes may play in lung diseases and infection. ..
  82. Ryan M, Akinbi H, Serrano A, Perez Gil J, Wu H, McCormack F, et al. Antimicrobial activity of native and synthetic surfactant protein B peptides. J Immunol. 2006;176:416-25 pubmed
    ..These results suggest that the role of endogenous SP-B in host defense may be limited; however, synthetic peptides derived from SP-B may be useful in the treatment of bacterial pneumonias. ..
  83. De Paepe M, Mao Q, Embree Ku M, Rubin L, Luks F. Fas/FasL-mediated apoptosis in perinatal murine lungs. Am J Physiol Lung Cell Mol Physiol. 2004;287:L730-42 pubmed
    ..The developmental time dependence of apoptosis-related events in the murine model should facilitate investigations of the regulation of perinatal pulmonary apoptotic gene expression. ..