Sftpb

Summary

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

Top Publications

  1. pmc Follistatin-like 1 (Fstl1) is a bone morphogenetic protein (BMP) 4 signaling antagonist in controlling mouse lung development
    Yan Geng
    Model Animal Research Center, Nanjing University, Nanjing 210061, China
    Proc Natl Acad Sci U S A 108:7058-63. 2011
  2. ncbi Involvement of Sonic hedgehog (Shh) in mouse embryonic lung growth and morphogenesis
    S Bellusci
    Department of Cell Biology, Vanderbilt University Medical Center, Nashville, Tennessee 37232 2175, USA
    Development 124:53-63. 1997
  3. pmc LPCAT1 regulates surfactant phospholipid synthesis and is required for transitioning to air breathing in mice
    James P Bridges
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio45229, USA
    J Clin Invest 120:1736-48. 2010
  4. pmc CARM1 is required for proper control of proliferation and differentiation of pulmonary epithelial cells
    Karen B O'Brien
    Harvard Stem Cell Institute and Center for Life Sciences, Harvard Medical School, Boston, MA 02115, USA
    Development 137:2147-56. 2010
  5. ncbi FGF-1 and FGF-7 induce distinct patterns of growth and differentiation in embryonic lung epithelium
    W V Cardoso
    The Pulmonary Center, Department of Medicine, Boston University School of Medicine, Massachusetts 02118, USA
    Dev Dyn 208:398-405. 1997
  6. pmc cAMP response element binding protein is required for differentiation of respiratory epithelium during murine development
    A Daniel Bird
    Department of Biochemistry and Molecular Biology, Monash University, Clayton, Victoria, Australia
    PLoS ONE 6:e17843. 2011
  7. pmc Epigenetic mechanisms modulate thyroid transcription factor 1-mediated transcription of the surfactant protein B gene
    YuXia Cao
    Pulmonary Center, Department of Medicine, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    J Biol Chem 285:2152-64. 2010
  8. ncbi Nmyc plays an essential role during lung development as a dosage-sensitive regulator of progenitor cell proliferation and differentiation
    Tadashi Okubo
    Department of Cell Biology, Duke University Medical Center, Durham, NC 27710, USA
    Development 132:1363-74. 2005
  9. pmc Eyes absent 1 (Eya1) is a critical coordinator of epithelial, mesenchymal and vascular morphogenesis in the mammalian lung
    Ahmed H K El-Hashash
    Developmental Biology and Regenerative Medicine Program, Saban Research Institute, Childrens Hospital Los Angeles, Keck School of Medicine of University of Southern California, 4650 Sunset Boulevard MS35, Los Angeles, CA 90027, USA
    Dev Biol 350:112-26. 2011
  10. ncbi TTF-1 phosphorylation is required for peripheral lung morphogenesis, perinatal survival, and tissue-specific gene expression
    Mario DeFelice
    Stazione Zoologica A Dohrn, Villa Comunale, 80121 Naples, Italy
    J Biol Chem 278:35574-83. 2003

Scientific Experts

  • Ahmed H K El-Hashash
  • M Ikegami
  • Yan Xu
  • Annamari Salminen
  • D R Prows
  • YuXia Cao
  • T E Weaver
  • Michael L Mucenski
  • Jingsong Xu
  • W V Cardoso
  • Tadashi Okubo
  • M T Stahlman
  • L P Rubin
  • D Picard
  • Angela Haczku
  • Monique E De Paepe
  • Hongwei Yu
  • Jeffrey A Whitsett
  • Katja Zscheppang
  • Li Yang
  • Yuzhen Zhang
  • Bing Xu
  • Edward E Morrisey
  • J A Whitsett
  • Christiane E L Dammann
  • Ross Ridsdale
  • Weiguo Shu
  • Huajing Wan
  • Henry T Akinbi
  • Min Min Lu
  • Changgong Li
  • A Daniel Bird
  • Yu Chih Hsu
  • Shengguo Li
  • Yan Geng
  • Andreas Schmiedl
  • Yiming Xing
  • Tina M Bunnell
  • James P Bridges
  • Karen B O'Brien
  • Washa Liu
  • Cheng Chen
  • Pablo Bringas
  • Wei Shi
  • Hui Chen
  • Ciara Field-Corbett
  • Zhonghua Hu
  • Jianping Sun
  • Katharine A Kevill
  • Hye Yeong Ha
  • Alysia Kern Lovgren
  • Jan Johansson
  • NaEun Cheong
  • N Mimura
  • Cindy J Bachurski
  • Michael L Fitzgerald
  • Linda O Tremblay
  • Hiroko Akei
  • Zea Borok
  • Parviz Minoo
  • Iwona Grad
  • Meng Chun Yang
  • Marnie A Ryan
  • Nuria Moreno-Barriuso
  • L E Fredenburgh
  • Fadel Tissir
  • Bernhard L Bader
  • Siew Lan Ang
  • Klaus H Kaestner
  • Igor O Nasonkin
  • Takayuki Ueno
  • Robin N N Han
  • Martin Post
  • Cheng Lun Na
  • L Lim
  • Ming O Li
  • Cong Yan
  • Dong Yan
  • Ralph Epaud
  • Jean C Clark
  • R H Costa
  • Hong Du
  • Kristin R Melton
  • Sussie Dalvin
  • Mario DeFelice
  • Juliana Johnson Conkright
  • Eleni Goshu
  • Albert Gründer
  • Kenzo Nakamura
  • M M Lu

Detail Information

Publications92

  1. pmc Follistatin-like 1 (Fstl1) is a bone morphogenetic protein (BMP) 4 signaling antagonist in controlling mouse lung development
    Yan Geng
    Model Animal Research Center, Nanjing University, Nanjing 210061, China
    Proc Natl Acad Sci U S A 108:7058-63. 2011
    ..Therefore, we provide in vivo and in vitro evidence to demonstrate that Fstl1 modulates lung development and alveolar maturation, in part, through BMP4 signaling...
  2. ncbi Involvement of Sonic hedgehog (Shh) in mouse embryonic lung growth and morphogenesis
    S Bellusci
    Department of Cell Biology, Vanderbilt University Medical Center, Nashville, Tennessee 37232 2175, USA
    Development 124:53-63. 1997
    ..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...
  3. pmc LPCAT1 regulates surfactant phospholipid synthesis and is required for transitioning to air breathing in mice
    James P Bridges
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio45229, USA
    J Clin Invest 120:1736-48. 2010
    ..Collectively, these data demonstrate that full LPCAT1 activity is required to achieve the levels of SatPC essential for the transition to air breathing...
  4. pmc CARM1 is required for proper control of proliferation and differentiation of pulmonary epithelial cells
    Karen B O'Brien
    Harvard Stem Cell Institute and Center for Life Sciences, Harvard Medical School, Boston, MA 02115, USA
    Development 137:2147-56. 2010
    ..These results demonstrate for the first time that CARM1 inhibits pulmonary cell proliferation and is required for proper differentiation of alveolar cells...
  5. ncbi FGF-1 and FGF-7 induce distinct patterns of growth and differentiation in embryonic lung epithelium
    W V Cardoso
    The Pulmonary Center, Department of Medicine, Boston University School of Medicine, Massachusetts 02118, USA
    Dev Dyn 208:398-405. 1997
    ..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...
  6. pmc cAMP response element binding protein is required for differentiation of respiratory epithelium during murine development
    A Daniel Bird
    Department of Biochemistry and Molecular Biology, Monash University, Clayton, Victoria, Australia
    PLoS ONE 6:e17843. 2011
    ..Together, these results demonstrate a crucial role for Creb1 activity for the development and differentiation of the conducting and distal lung epithelium...
  7. pmc Epigenetic mechanisms modulate thyroid transcription factor 1-mediated transcription of the surfactant protein B gene
    YuXia Cao
    Pulmonary Center, Department of Medicine, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    J Biol Chem 285:2152-64. 2010
    ..Surfactant protein B (Sftpb) is a developmentally regulated lung epithelial gene critical for lung function...
  8. ncbi Nmyc plays an essential role during lung development as a dosage-sensitive regulator of progenitor cell proliferation and differentiation
    Tadashi Okubo
    Department of Cell Biology, Duke University Medical Center, Durham, NC 27710, USA
    Development 132:1363-74. 2005
    ..We propose a model in which Nmyc is essential in the developing lung for maintaining a distal population of undifferentiated, proliferating progenitor cells...
  9. pmc Eyes absent 1 (Eya1) is a critical coordinator of epithelial, mesenchymal and vascular morphogenesis in the mammalian lung
    Ahmed H K El-Hashash
    Developmental Biology and Regenerative Medicine Program, Saban Research Institute, Childrens Hospital Los Angeles, Keck School of Medicine of University of Southern California, 4650 Sunset Boulevard MS35, Los Angeles, CA 90027, USA
    Dev Biol 350:112-26. 2011
    ..We conclude, therefore, that Eya1 function is critical for proper coordination of lung epithelial, mesenchymal and vascular development...
  10. ncbi TTF-1 phosphorylation is required for peripheral lung morphogenesis, perinatal survival, and tissue-specific gene expression
    Mario DeFelice
    Stazione Zoologica A Dohrn, Villa Comunale, 80121 Naples, Italy
    J Biol Chem 278:35574-83. 2003
    ..Direct and indirect transcriptional targets of TTF-1 were identified that are likely to play important roles in lung formation and function...
  11. ncbi Defective heparan sulfate biosynthesis and neonatal lethality in mice lacking N-deacetylase/N-sulfotransferase-1
    M Ringvall
    Departments of Cell and Molecular Biology and Medical Biochemistry and Microbiology, Uppsala University, S 751 23 Uppsala, Sweden
    J Biol Chem 275:25926-30. 2000
    ..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...
  12. ncbi Wnt5a participates in distal lung morphogenesis
    Changgong Li
    Department of Pediatrics, Women s and Children s Hospital, Los Angeles, CA 90033, USA
    Dev Biol 248:68-81. 2002
    ..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...
  13. ncbi The TTF-1/TAP26 complex differentially modulates surfactant protein-B (SP-B) and -C (SP-C) promoters in lung cells
    Meng Chun Yang
    Department of Internal Medicine, Pulmonary and Critical Care Medicine, UT Southwestern Medical Center, Dallas, TX 75390 9034, USA
    Biochem Biophys Res Commun 344:484-90. 2006
    ..Thus, a dimerized complex structure was needed for advanced promoter activity. This result also provided a molecular mechanism by which both the SP-B and SP-C promoters could be differentially regulated by the same complex...
  14. ncbi Sequence, ontogeny, and cellular localization of murine surfactant protein B mRNA
    M A D'Amore-Bruno
    Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229
    Am J Physiol 262:L40-7. 1992
    ..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...
  15. pmc The Hsp90 cochaperone p23 is essential for perinatal survival
    Iwona Grad
    Departement de Biologie Cellulaire, Universite de Geneve, Sciences III, 1211 Geneve 4, Switzerland
    Mol Cell Biol 26:8976-83. 2006
    ..Thus, p23 contributes a nonredundant, temporally restricted, and tissue-specific function during mouse development...
  16. pmc Compound genetic ablation of nidogen 1 and 2 causes basement membrane defects and perinatal lethality in mice
    Bernhard L Bader
    Department of Dermatology, University of Cologne, 50924 Cologne, Germany
    Mol Cell Biol 25:6846-56. 2005
    ..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...
  17. ncbi beta-Catenin is required for specification of proximal/distal cell fate during lung morphogenesis
    Michael L Mucenski
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    J Biol Chem 278:40231-8. 2003
    ..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...
  18. pmc Foxa2 is required for transition to air breathing at birth
    Huajing Wan
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center and University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
    Proc Natl Acad Sci U S A 101:14449-54. 2004
    ..Foxa2 regulates a complex pulmonary program of epithelial cell maturation required for transition to air breathing at birth...
  19. ncbi SP-B deficiency causes respiratory failure in adult mice
    Kristin R Melton
    Cincinnati Children s Hospital Medical Center, Division of Pulmonary Biology, 3333 Burnet Ave, Cincinnati, OH 45229 3039, USA
    Am J Physiol Lung Cell Mol Physiol 285:L543-9. 2003
    ..Reduction of alveolar SP-B content causes surfactant dysfunction and respiratory failure, indicating that SP-B is required for postnatal lung function...
  20. ncbi Absence of heme oxygenase-1 expression in the lung parenchyma exacerbates endotoxin-induced acute lung injury and decreases surfactant protein-B levels
    L E Fredenburgh
    Division of Pulmonary and Critical Medicine, Department of Medicine, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Cell Mol Biol (Noisy-le-grand) 51:513-20. 2005
    ..These findings have implications for our understanding of the pathophysiology of ARDS and may guide future therapeutic strategies...
  21. ncbi Thyroid transcription factor-1, hepatocyte nuclear factor-3beta, surfactant protein B, C, and Clara cell secretory protein in developing mouse lung
    L Zhou
    Children s Hospital Medical Center, Division of Pulmonary Biology, Cincinnati, Ohio 45229 3039, USA
    J Histochem Cytochem 44:1183-93. 1996
    ..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...
  22. pmc Delayed embryonic development and impaired cell growth and survival in Actg1 null mice
    Tina M Bunnell
    Department of Biochemistry, Molecular Biology and Biophysics, University of Minnesota, Minneapolis, 55455, USA
    Cytoskeleton (Hoboken) 67:564-72. 2010
    ..Since the total amount of actin protein was maintained in Actg1(-/-) cells, our data suggests a distinct requirement for gamma(cyto)-actin in cell growth and survival...
  23. pmc p21(CIP1) and p57(KIP2) control muscle differentiation at the myogenin step
    P Zhang
    Howard Hughes Medical Institute, Baylor College of Medicine, Houston, Texas 77030 USA
    Genes Dev 13:213-24. 1999
    ....
  24. doi Regulation of surfactant protein B gene expression in bone marrow-derived cells
    Ciara Field-Corbett
    Institute of Immunology, Biology Department, National University of Ireland Maynooth, Ireland
    Stem Cells 27:662-9. 2009
    ..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...
  25. ncbi Processing of pulmonary surfactant protein B by napsin and cathepsin H
    Takayuki Ueno
    Cancer Center Karolinska, Department of Oncology Pathology, Karolinska Institute and Hospital, SE 171 76 Stockholm, Sweden
    J Biol Chem 279:16178-84. 2004
    ..These results suggest that napsin, cathepsin H, and at least one other enzyme are involved in maturation of the biologically active SP-B peptide...
  26. ncbi Tumor necrosis factor-alpha inhibits surfactant protein C gene transcription
    C J Bachurski
    Children s Hospital Medical Center, Division of Pulmonary Biology, Cincinnati, Ohio 45229 3039, USA
    J Biol Chem 270:19402-7. 1995
    ..Inhibition of surfactant protein C gene transcription by TNF-alpha may contribute to the abnormalities of surfactant homeostasis associated with pulmonary injury and infection...
  27. pmc Mesenchymal nuclear factor I B regulates cell proliferation and epithelial differentiation during lung maturation
    Yu Chih Hsu
    Department of Biochemistry, Developmental Genomics Group, Center of Excellence in Bioinformatics and Life Science, State University of New York at Buffalo, 701 Ellicott St Buffalo, NY 14203, USA
    Dev Biol 354:242-52. 2011
    ..Our data indicate that mesenchymal Nfib regulates both mesenchymal and epithelial cell proliferation through multiple pathways and that mesenchymal NFI-B-mediated signals are essential for the maturation of distal lung epithelium...
  28. ncbi Alterations in alveolar epithelium differentiation and vasculogenesis in lungs of LIF/IGF-I double deficient embryos
    Nuria Moreno-Barriuso
    Instituto de Biologia Molecular y Celular del Cancer, Consejo Superior de Investigaciones Cientificas Universidad de Salamanca, Salamanca, Spain
    Dev Dyn 235:2040-50. 2006
    ..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...
  29. pmc Surfactant protein B propeptide contains a saposin-like protein domain with antimicrobial activity at low pH
    Li Yang
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, OH 45229 3039, USA
    J Immunol 184:975-83. 2010
    ..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...
  30. ncbi mnd2: a new mouse model of inherited motor neuron disease
    J M Jones
    Department of Human Genetics, University of Michigan Medical School, Ann Arbor 48109
    Genomics 16:669-77. 1993
    ..This new animal model will be of value for identification of a genetic defect responsible for motor neuron disease and for evaluation of new therapies...
  31. ncbi Use of knockout mice to study surfactant protein structure and function
    T E Weaver
    Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Biol Neonate 76:15-8. 1999
    ..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...
  32. ncbi HNF-3/forkhead homologue-4 influences lung morphogenesis and respiratory epithelial cell differentiation in vivo
    J W Tichelaar
    Division of Pulmonary Biology, Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, Ohio, 45229 3039, USA
    Dev Biol 213:405-17. 1999
    ..Ectopic expression of HFH-4 in developing mouse lung altered epithelial cell differentiation and morphology, restricting the expression of markers typical of nonciliated cells of the distal lung parenchyma...
  33. ncbi Surface tension influences cell shape and phagocytosis in alveolar macrophages
    Hiroko Akei
    Cincinnati Children s Hospital, Division of Pulmonary Biology, University of Cincinnati, OH 45229 3039, USA
    Am J Physiol Lung Cell Mol Physiol 291:L572-9. 2006
    ..Surface tension was regulated in vivo by conditionally expressing surfactant protein (SP)-B in Sftpb-/- mice...
  34. pmc Neuregulin receptor ErbB4 functions as a transcriptional cofactor for the expression of surfactant protein B in the fetal lung
    Katja Zscheppang
    Department of Pediatrics, Hannover Medical School, Hannover, Germany
    Am J Respir Cell Mol Biol 45:761-7. 2011
    ..We hypothesized that in the fetal lung, ErbB4 functions as a transcriptional regulator for surfactant protein B (Sftpb), the most critical surfactant protein gene...
  35. ncbi Defects in pulmonary vasculature and perinatal lung hemorrhage in mice heterozygous null for the Forkhead Box f1 transcription factor
    V V Kalinichenko
    University of Illinois at Chicago, College of Medicine, Department of Molecular Genetics, 900 S Ashland Ave, Chicago, IL 60607 7170, USA
    Dev Biol 235:489-506. 2001
    ..Reduction in the level of Foxf1 caused neonatal pulmonary hemorrhage and abnormalities in alveologenesis, implicating this transcription factor in the regulation of mesenchyme-epithelial interaction critical for lung morphogenesis...
  36. pmc Transcriptional programs controlling perinatal lung maturation
    Yan Xu
    The Perinatal Institute and Section of Neonatology, Perinatal and Pulmonary Biology, University of Cincinnati, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio, USA
    PLoS ONE 7:e37046. 2012
    ..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...
  37. pmc Estrogen-induced upregulation of Sftpb requires transcriptional control of neuregulin receptor ErbB4 in mouse lung type II epithelial cells
    Katja Zscheppang
    Department of Pediatrics, Hannover Medical School, 30625 Hannover, Germany
    Biochim Biophys Acta 1813:1717-27. 2011
    ..We hypothesized that estrogen signals its effect on surfactant protein B (Sftpb) expression through interactions of ERβ and ErbB4...
  38. pmc Foxn4 influences alveologenesis during lung development
    Shengguo Li
    Center for Advanced Biotechnology and Medicine and Department of Pediatrics, UMDNJ Robert Wood Johnson Medical School, Piscataway, New Jersey 08854, USA
    Dev Dyn 240:1512-7. 2011
    ..may result from decreased platelet-derived growth factor-A (PDGFA) signaling and reduced surfactant protein B (SFTPB) expression...
  39. ncbi ABCA3 is critical for lamellar body biogenesis in vivo
    NaEun Cheong
    Department of Physiology, University of Pennsylvania School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 282:23811-7. 2007
    ..Collectively, these findings strongly suggest that ABCA3 is necessary for lamellar body biogenesis, surfactant protein-B processing, and lung development late in gestation...
  40. pmc cPGES/p23 is required for glucocorticoid receptor function and embryonic growth but not prostaglandin E2 synthesis
    Alysia Kern Lovgren
    University of North Carolina, Department of Genetics, Chapel Hill, NC 27599, USA
    Mol Cell Biol 27:4416-30. 2007
    ....
  41. ncbi GATA and Nkx factors synergistically regulate tissue-specific gene expression and development in vivo
    Yuzhen Zhang
    Department of Medicine and University of Pennsylvania, Philadelphia, PA 19104, USA
    Development 134:189-98. 2007
    ....
  42. ncbi ABCA3 inactivation in mice causes respiratory failure, loss of pulmonary surfactant, and depletion of lung phosphatidylglycerol
    Michael L Fitzgerald
    Lipid Metabolism Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
    J Lipid Res 48:621-32. 2007
    ..They also demonstrate the utility of the Abca3 null mouse as a model for a devastating human disease...
  43. ncbi Respiratory distress and neonatal lethality in mice lacking Golgi alpha1,2-mannosidase IB involved in N-glycan maturation
    Linda O Tremblay
    McGill Cancer Centre, McGill University, Montreal, Quebec, Canada
    J Biol Chem 282:2558-66. 2007
    ..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...
  44. ncbi Structure and function of the mouse surfactant protein B gene
    M A Bruno
    Am J Physiol 268:section L following t of c. 1995
  45. doi ErbB4 regulates the timely progression of late fetal lung development
    Washa Liu
    Division of Newborn Medicine, Floating Hospital for Children at Tufts Medical Center, Boston, MA 02111, USA
    Biochim Biophys Acta 1803:832-9. 2010
    ..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...
  46. ncbi A role for macrophage migration inhibitory factor in the neonatal respiratory distress syndrome
    Katharine A Kevill
    Department of Pediatrics, Yale University School of Medicine, New Haven, CT 06520, USA
    J Immunol 180:601-8. 2008
    ..03). This study demonstrates for the first time a role for MIF in lung maturation, and supports a protective role for MIF in newborn lung disease...
  47. ncbi Mapping of two genes encoding members of a distinct subfamily of MAX interacting proteins: MAD to human chromosome 2 and mouse chromosome 6, and MXI1 to human chromosome 10 and mouse chromosome 19
    S Edelhoff
    Department of Pathology, University of Washington, Seattle 98195
    Oncogene 9:665-8. 1994
    ..Human chromosomes 2p13 and 10q25 have been involved in specific tumors where the role of Mad and Mxi1 can now be investigated...
  48. pmc Signaling via Alk5 controls the ontogeny of lung Clara cells
    Yiming Xing
    Department of Pediatrics, Division of Neonatology, University of Southern California, Keck School of Medicine, Los Angeles, CA 90033, USA
    Development 137:825-33. 2010
    ..Thus, the signaling pathway by which TGFbeta/ALK5 regulates Clara cell differentiation may entail inhibition of Pten expression, which in turn activates ERK and AKT phosphorylation...
  49. doi NDST1-dependent heparan sulfate regulates BMP signaling and internalization in lung development
    Zhonghua Hu
    State Key Laboratory of Molecular Biology, Institute of Biochemistry and Cell Biology, Shanghai Institute for Biological Sciences, Chinese Academy of Sciences, 200031 Shanghai, China
    J Cell Sci 122:1145-54. 2009
    ..The results suggest that NDST1-dependent HS is essential for proper functioning of BMP in embryonic lung development...
  50. pmc Pulmonary surfactant surface tension influences alveolar capillary shape and oxygenation
    Machiko Ikegami
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
    Am J Respir Cell Mol Biol 41:433-9. 2009
    ..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 ..
  51. ncbi Intersubunit disulfide bridge is not required for the protective role of SP-B against lung inflammation
    Machiko Ikegami
    Division of Pulmonary Biology, Cincinnati Children s Hospital, Cincinnati, Ohio 45229 3039, USA
    J Appl Physiol (1985) 93:505-11. 2002
    ..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...
  52. pmc A Gata6-Wnt pathway required for epithelial stem cell development and airway regeneration
    Yuzhen Zhang
    Department of Medicine, University of Pennsylvania, 956 BRB II III, 421 Curie Boulevard, Philadelphia, Pennsylvania 19104, USA
    Nat Genet 40:862-70. 2008
    ..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...
  53. pmc Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice
    J C Clark
    Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
    Proc Natl Acad Sci U S A 92:7794-8. 1995
    ..Ablation of the SP-B gene disrupts the routing, storage, and function of surfactant phospholipids and proteins, causing respiratory failure at birth...
  54. pmc Cited2 is required for fetal lung maturation
    Bing Xu
    Department of Pharmacology, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA
    Dev Biol 317:95-105. 2008
    ..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...
  55. pmc Smad1 and its target gene Wif1 coordinate BMP and Wnt signaling activities to regulate fetal lung development
    Bing Xu
    Developmental Biology and Regenerative Medicine Program, Children s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027, USA
    Development 138:925-35. 2011
    ..This suggests a novel regulatory loop of Bmp4-Smad1-Wif1-Wnt/β-catenin in coordinating BMP and Wnt pathways to control fetal lung development...
  56. doi Morphogenetic lung defects of JSAP1-deficient embryos proceeds via the disruptions of the normal expressions of cytoskeletal and chaperone proteins
    Hye Yeong Ha
    Division of Nano Sciences and Brain Disease Research Institute, Ewha Womans University, Seoul, Republic of Korea
    Proteomics 8:1071-80. 2008
    ....
  57. pmc Prenatal lung epithelial cell-specific abrogation of Alk3-bone morphogenetic protein signaling causes neonatal respiratory distress by disrupting distal airway formation
    Jianping Sun
    Developmental Biology Program, Childrens Hospital Los Angeles, 4650 Sunset Blvd, MS 35, Los Angeles, CA 90027, USA
    Am J Pathol 172:571-82. 2008
    ..Therefore, our data suggest that deficiency of appropriate BMP signaling in lung epithelial cells results in prenatal lung malformation, neonatal atelectasis, and respiratory failure...
  58. ncbi Surfactant lipid synthesis and lamellar body formation in glycogen-laden type II cells
    Ross Ridsdale
    Canadian Institutes of Health Research Group in Lung Development, Hospital for Sick Children Research Institute, and Institute of Medical Sciences, University of Toronto, Toronto, Ontario, M5G 1X8, Canada
    Am J Physiol Lung Cell Mol Physiol 287:L743-51. 2004
    ....
  59. ncbi Antimicrobial activity of native and synthetic surfactant protein B peptides
    Marnie A Ryan
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, and University of Cincinnati College of Medicine, OH 45229 3039, USA
    J Immunol 176:416-25. 2006
    ..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...
  60. ncbi Genetic susceptibility to nickel-induced acute lung injury
    Daniel R Prows
    Department of Environmental Health, University of Cincinnati, Cincinnati, OH 45267 0056, USA
    Chemosphere 51:1139-48. 2003
    ..Together, results from QTL and microarray analyses of nickel-induced acute lung injury survival allowed us to generate a short list of candidate genes...
  61. ncbi The roles of surfactant proteins A and D in innate immunity
    P R Lawson
    Department of Biochemistry, Oxford University, England
    Immunol Rev 173:66-78. 2000
    ..Linkage and polymorphism analysis is explaining the role these genes may play in lung diseases and infection...
  62. ncbi Surfactant protein B inhibits endotoxin-induced lung inflammation
    Ralph Epaud
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    Am J Respir Cell Mol Biol 28:373-8. 2003
    ....
  63. ncbi Wnt7b regulates mesenchymal proliferation and vascular development in the lung
    Weiguo Shu
    Department of Medicine and the Molecular Cardiology Research Center, University of Pennsylvania, Philadelphia, PA 19104, USA
    Development 129:4831-42. 2002
    ..These defects lead to rupture of the major vessels and hemorrhage in the lungs after birth. These results demonstrate that Wnt7b signaling is required for proper lung mesenchymal growth and vascular development...
  64. ncbi Endogenous and exogenous glucocorticoid regulation of ENaC mRNA expression in developing kidney and lung
    Kenzo Nakamura
    Department of Internal Medicine, University of Iowa College of Medicine and Veterans Affairs Medical Center, Iowa City 52242, USA
    Am J Physiol Cell Physiol 283:C762-72. 2002
    ....
  65. ncbi Ovine surfactant protein cDNAs: use in studies on fetal lung growth and maturation after prolonged hypoxemia
    G A Braems
    Department of Obstetrics and Gynaecology, University of Western Ontario, London, Ontario N6A 5A5
    Am J Physiol Lung Cell Mol Physiol 278:L754-64. 2000
    ..We conclude that these data suggest that mild prolonged fetal hypoxia produces alterations that could affect fetal cellular differentiation early in gestation and can induce changes consistent with lung maturation closer to term...
  66. pmc Sim2 mutants have developmental defects not overlapping with those of Sim1 mutants
    Eleni Goshu
    Department of Embryology, Carnegie Institution of Washington, Baltimore, Maryland 21210, USA
    Mol Cell Biol 22:4147-57. 2002
    ..The temporal and spatial expression patterns of Sim2 in these skeletal elements suggest that Sim2 regulates their growth and/or integrity...
  67. ncbi Nuclear factor I-B (Nfib) deficient mice have severe lung hypoplasia
    Albert Gründer
    Institut fur Biologie III, Albert Ludwigs Universitat, Freiburg, Germany
    Mech Dev 112:69-77. 2002
    ..Our study demonstrates that Nfib is essential for normal lung development, and suggests that it could be involved in the pathogenesis of respiratory distress syndromes in humans...
  68. ncbi Deficiency of SP-B reveals protective role of SP-C during oxygen lung injury
    Machiko Ikegami
    Children s Hospital Medical Center, Division of Pulmonary Biology, Cincinnati, Ohio 45229 3039, USA
    J Appl Physiol (1985) 92:519-26. 2002
    ..Genetically decreased levels of SP-B combined with superimposed O(2)-induced injury reveals the distinct contribution of SP-C to pulmonary function in vivo...
  69. ncbi Overexpression of surfactant protein-C mature peptide causes neonatal lethality in transgenic mice
    Juliana Johnson Conkright
    Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Am J Respir Cell Mol Biol 26:85-90. 2002
    ..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...
  70. ncbi The bone morphogenic protein antagonist gremlin regulates proximal-distal patterning of the lung
    M M Lu
    Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Dev Dyn 222:667-80. 2001
    ..These data suggest that gremlin plays an important role in lung morphogenesis by regulating the proximal-distal patterning of the lung during development...
  71. ncbi Haploinsufficiency of the forkhead gene Foxf1, a target for sonic hedgehog signaling, causes lung and foregut malformations
    M Mahlapuu
    Department of Molecular Biology, Goteborg University, The Lundberg Laboratory, Box 462, SE 405 30 Goteborg, Sweden
    Development 128:2397-406. 2001
    ..Fibroblast growth factor (FGF) 10 and FGF7 both decrease Foxf1 expression and we speculate that this is mediated by transcriptional activation of epithelial Bmp4 (in the case of FGF10) and by inhibition of Shh expression for FGF7...
  72. ncbi Expression of Netrin-1 and its two receptors DCC and UNC5H2 in the developing mouse lung
    Sussie Dalvin
    Department of Pediatrics, Massachusetts General Hospital for Children, Harvard Medical School, Boston, MA 02114, USA
    Gene Expr Patterns 3:279-83. 2003
    ..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...
  73. ncbi Peroxisome proliferator-activated receptor gamma and ligands inhibit surfactant protein B gene expression in the lung
    Li Yang
    Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    J Biol Chem 278:36841-7. 2003
    ..The studies support the concept that surfactant protein B homeostasis is influenced by neutral lipid metabolites in the lung...
  74. ncbi Surfactant protein-B-deficient mice are susceptible to hyperoxic lung injury
    K Tokieda
    Division of Neonatology and Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Am J Respir Cell Mol Biol 21:463-72. 1999
    ..These data support the concept that SP-B plays an important protective role in the lung...
  75. ncbi Tumor necrosis factor-alpha decreases surfactant protein B mRNA in murine lung
    G S Pryhuber
    Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    Am J Physiol 270:L714-21. 1996
    ..TNF-alpha-mediated decrease in SP gene expression may contribute to the surfactant dysfunction and atelectasis observed in inflammatory lung diseases...
  76. ncbi Protocadherin Celsr3 is crucial in axonal tract development
    Fadel Tissir
    Developmental Neurobiology Unit, University of Louvain Medical School, 73, Avenue Mounier, Box DENE7382, B 1200 Brussels, Belgium
    Nat Neurosci 8:451-7. 2005
    ..Celsr3 and Fzd3 are expressed together during brain development and may act in synergy. Thus, a genetic pathway analogous to the one that controls PCP is key in the development of the axonal blueprint...
  77. ncbi Normal lung development in RAIG1-deficient mice despite unique lung epithelium-specific expression
    Jingsong Xu
    Harvard Medical School, Brigham and Women s Hospital, 75 Francis St, Boston, MA 02115, USA
    Am J Respir Cell Mol Biol 32:381-7. 2005
    ..Analysis of other family members demonstrated some overlapping embryonic expression of RAIG3 mRNA that could have led to functional redundancy in the single RAIG1 null mutant mouse...
  78. ncbi Compensatory roles of Foxa1 and Foxa2 during lung morphogenesis
    Huajing Wan
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center and the University of Cincinnati College of Medicine, Cincinnati, Ohio 45229 3039, USA
    J Biol Chem 280:13809-16. 2005
    ..Foxa family members regulate signaling and transcriptional programs required for morphogenesis and cell differentiation during formation of the lung...
  79. ncbi Pituitary hypoplasia and respiratory distress syndrome in Prop1 knockout mice
    Igor O Nasonkin
    Department of Human Genetics, University of Michigan Medical School, Ann Arbor, MI 48109, USA
    Hum Mol Genet 13:2727-35. 2004
    ..Prop1-null mice are an excellent model for MPHD and may be useful for testing the efficacy of pharmaceutical intervention for neonatal respiratory distress...
  80. ncbi Chromosomal mapping of five mouse G protein gamma subunits
    G B Downes
    Department of Anesthesiology, Washington University School of Medicine, St Louis, Missouri, 63110, USA
    Genomics 57:173-6. 1999
    ..Combined with previous mapping studies, these data indicate that, with the possible exception of gamma1 and gamma11, the G protein gamma subunit genes are well dispersed within the mouse and human genomes...
  81. pmc Dach1 mutant mice bear no gross abnormalities in eye, limb, and brain development and exhibit postnatal lethality
    R J Davis
    Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
    Mol Cell Biol 21:1484-90. 2001
    ..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...
  82. pmc Hyperactive Wnt signaling changes the developmental potential of embryonic lung endoderm
    Tadashi Okubo
    Department of Cell Biology, Duke University Medical Center, Durham, NC 27710, USA
    J Biol 3:11. 2004
    ..The full range of effects of this pathway, which includes the transcription factor Lef1, has not been explored, however...
  83. ncbi Defective lung vascular development and fatal respiratory distress in endothelial NO synthase-deficient mice: a model of alveolar capillary dysplasia?
    Robin N N Han
    Terrence Donnelly Heart Center, Division of Cardiology, St Michael s Hospital and the Department of Medicine, University of Toronto, Toronto, Canada
    Circ Res 94:1115-23. 2004
    ..We conclude that eNOS plays a previously unrecognized role in lung development, which may have relevance for clinical syndromes of neonatal respiratory distress...
  84. pmc Late gestational lung hypoplasia in a mouse model of the Smith-Lemli-Opitz syndrome
    Hongwei Yu
    Division of Endocrinology, Diabetes and Medical Genetics, Medical University of South Carolina, STR 541, 114 Doughty Street, Charleston, SC 29403, USA
    BMC Dev Biol 4:1. 2004
    ..Although they have a number of biochemical and structural abnormalities, one cause of death is from apparent respiratory failure due to developmental pulmonary abnormalities...
  85. ncbi Phosphatidylserine receptor is required for clearance of apoptotic cells
    Ming O Li
    Section of Immunobiology, Yale University School of Medicine, New Haven, CT 06520, USA
    Science 302:1560-3. 2003
    ..These data demonstrate a critical role for PSR in early stages of mammalian organogenesis and suggest that this receptor may be involved in respiratory distress syndromes and congenital brain malformations...
  86. ncbi Structure and function of the mouse surfactant protein B gene
    M A Bruno
    Children s Hospital Research Foundation, Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229
    Am J Physiol 268:L381-9. 1995
    ..The 5' flanking region of the murine SP-B contains cell-specific cis-active elements that regulate SP-B expression in the respiratory epithelium...
  87. ncbi Foxp2 and Foxp1 cooperatively regulate lung and esophagus development
    Weiguo Shu
    Cardiovascular Institute, University of Pennsylvania, 956 BRB II III, 421 Curie Blvd, Philadelphia, PA 19104, USA
    Development 134:1991-2000. 2007
    ....
  88. ncbi Molecular characterization of a novel human gene, SEC13R, related to the yeast secretory pathway gene SEC13, and mapping to a conserved linkage group on human chromosome 3p24-p25 and mouse chromosome 6
    A Swaroop
    Department of Ophthalmology, University of Michigan, Ann Arbor 48109 0618
    Hum Mol Genet 3:1281-6. 1994
    ..The mouse Sec13r gene was mapped to the conserved linkage group on chromosome 6 that corresponds to human chromosome 3p24-p25...
  89. ncbi Aberrant quality control in the endoplasmic reticulum impairs the biosynthesis of pulmonary surfactant in mice expressing mutant BiP
    N Mimura
    Department of Anesthesiology, Chiba University Graduate School of Medicine, 1 8 1 Inohana, Chuo Ku, Chiba City, Chiba, Japan
    Cell Death Differ 14:1475-85. 2007
    ..Moreover, failure of this adaptive mechanism may increase pulmonary susceptibility to environmental insults, such as hypoxia and ischemia, ultimately leading to neonatal respiratory failure...
  90. ncbi Arrested pulmonary alveolar cytodifferentiation and defective surfactant synthesis in mice missing the gene for parathyroid hormone-related protein
    Lewis P Rubin
    Department of Pediatrics, Brown Medical School and Women and Infants Hospital, Providence, Rhode Island 02905 2499, USA
    Dev Dyn 230:278-89. 2004
    ....
  91. ncbi An enhancer region determines hSP-B gene expression in bronchiolar and ATII epithelial cells in transgenic mice
    Li Yang
    Division of Pulmonary Biology, Graduate Program for Molecular and Developmental Biology, and Division of Human Genetics, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Am J Physiol Lung Cell Mol Physiol 284:L481-8. 2003
    ..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...

Research Grants25

  1. Genetic analysis of hyperoxia-induced acute lung injury
    DANIEL PROWS; Fiscal Year: 2007
    ..abstract_text> ..
  2. Surfactant-Protein Innate Immunity in an Asthma Model
    Angela Haczku; Fiscal Year: 2007
    ..The potential opportunity to interfere with allergic sensitization by SP-D treatment bears high clinical significance. ..
  3. Regulation of Inflammation in Asthma by Fas Ligand
    Angela Haczku; Fiscal Year: 2009
    ..Finally, in order to help support the hypothesis that pro-apoptotic FasL activity characterized in human airway cells is truly physiologic, we will perform challenge studies in mice under FasL neutralizing conditions. ..
  4. Genetic Analysis of Hyperoxia Induced Acute Lung Injury
    DANIEL PROWS; Fiscal Year: 2009
    ..The primary objective of this application is to set the stage for identifying the major gene(s) controlling survival by generating and testing genetically-refined mouse models containing susceptibility alleles in a fixed background. ..
  5. Role of C/EBPalpha in Cytoprotection and Recovery from Lung Injury
    Machiko Ikegami; Fiscal Year: 2010
    ..Furthermore, the molecular mechanisms, in particular the signaling pathways regulating Cebpa, of recombinant human FGF-7 treatment for acute lung injury will be determined. ..
  6. Genetic Analysis of Hyperoxia Induced Acute Lung Injury
    Daniel R Prows; Fiscal Year: 2010
    ..The primary objective of this application is to set the stage for identifying the major gene(s) controlling survival by generating and testing genetically-refined mouse models containing susceptibility alleles in a fixed background. ..
  7. ROLE OF SURFACTANT PROTEIN D IN SURFACTANT HOMEOSTASIS
    Machiko Ikegami; Fiscal Year: 2006
    ..The intended studies will provide fundamental insights into the role of SP-D in the protection of the lung, as well as into the basic mechanisms determining surfactant homeostasis. ..
  8. ROLE OF SURFACTANT PROTEIN D IN SURFACTANT HOMEOSTASIS
    Machiko Ikegami; Fiscal Year: 2002
    ..The goal is to understand the role of SP-D in the cellular and/or structural mechanisms involved in independent regulation of phospholipid and surfactant protein homeostasis. ..
  9. Mechanisms of social-stress enhanced allergic airway response in a mouse model
    Angela Haczku; Fiscal Year: 2010
    ..2. Elucidation of the cellular and molecular pathways of the glucocorticoid action on immune cell function and epithelial cell SP-D production in stress-induced exacerbation of the inflammatory airway response. ..