SFTPB

Summary

Gene Symbol: SFTPB
Description: surfactant protein B
Alias: PSP-B, SFTB3, SFTP3, SMDP1, SP-B, pulmonary surfactant-associated protein B, 18 kDa pulmonary-surfactant protein, 6 kDa protein, pulmonary surfactant-associated proteolipid SPL(Phe)
Species: human

Top Publications

  1. pmc Polymorphic variation in surfactant protein B is associated with COPD exacerbations
    M G Foreman
    Channing Laboratory, Brigham and Women s Hospital, Boston, MA 02115, USA
    Eur Respir J 32:938-44. 2008
  2. 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
  3. ncbi Structure and organization of the gene encoding human pulmonary surfactant proteolipid SP-B
    T J Pilot-Matias
    Corporate Molecular Biology, Abbott Laboratories, IL 60064
    DNA 8:75-86. 1989
  4. pmc A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds
    L M Nogee
    Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287
    J Clin Invest 93:1860-3. 1994
  5. ncbi Intracellular localization of processing events in human surfactant protein B biosynthesis
    A Korimilli
    Division of Neonatology, University of Pennsylvania School of Medicine, Department of Pediatrics, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 275:8672-9. 2000
  6. pmc Differences in N-linked glycosylation between human surfactant protein-B variants of the C or T allele at the single-nucleotide polymorphism at position 1580: implications for disease
    Guirong Wang
    Department of Cellular and Molecular Physiology, The Pennsylvania State University, College of Medicine, Hershey, PA 17033, USA
    Biochem J 369:179-84. 2003
  7. ncbi Nitric oxide inhibits surfactant protein B gene expression in lung epithelial cells
    Darrell Salinas
    Department of Molecular Biology, University of Texas Health Center at Tyler, Tyler, Texas 75708 3154, USA
    Am J Physiol Lung Cell Mol Physiol 285:L1153-65. 2003
  8. ncbi Surfactant protein B in type II pneumocytes and intra-alveolar surfactant forms of human lungs
    Frank Brasch
    Department of Anatomy, University of Gottingen, Gottingen, Germany
    Am J Respir Cell Mol Biol 30:449-58. 2004
  9. ncbi Involvement of napsin A in the C- and N-terminal processing of surfactant protein B in type-II pneumocytes of the human lung
    Frank Brasch
    Department of Anatomy, University of Gottingen, Kreuzbergring 36, D 37075 Gottingen, Germany
    J Biol Chem 278:49006-14. 2003
  10. ncbi Dysfunction of pulmonary surfactant in chronically ventilated premature infants
    Jeffrey D Merrill
    Neonatology, Department of Pediatrics, University of Pennsylvania School of Medicine, USA
    Pediatr Res 56:918-26. 2004

Research Grants

  1. OZONE EFFECTS ON FUNCTION OF SURFACTANT PROTEIN VARIANTS
    Joanna Floros; Fiscal Year: 2010
  2. Growth Factor Protection in Acute Lung Injury
    GEORGE LEIKAUF; Fiscal Year: 2009
  3. Asthma Candidate Genes in Alpha 1-Antitrypsin Deficiency
    Dawn DeMeo; Fiscal Year: 2007
  4. The Role of surfactant protein genetic variants in cyst*
    Joanna Floros; Fiscal Year: 2005
  5. In Vivo Metabolism of Pulmonary Surfactant in Infants
    Aaron Hamvas; Fiscal Year: 2005
  6. EPIDEMIOLOGY OF SURFACTANT PROTEIN-B DEFICIENCY
    FRANCIS SESSIONS COLE; Fiscal Year: 2013
  7. SURFACTANT PATHWAY GENE VARIANTS AMONG INFANTS WITH RESPIRATORY DISTRESS SYNDROME
    JENNIFER WAMBACH; Fiscal Year: 2013
  8. Nilufer Ertekin-Taner; Fiscal Year: 2016
  9. SHH and Alveolar Formation
    Jeffrey Whitsett; Fiscal Year: 2006
  10. PERINATAL EXPRESSION OF SURFACTANT PROTEIN GENE SP-B
    Jeffrey Whitsett; Fiscal Year: 2007

Scientific Experts

Detail Information

Publications137 found, 100 shown here

  1. pmc Polymorphic variation in surfactant protein B is associated with COPD exacerbations
    M G Foreman
    Channing Laboratory, Brigham and Women s Hospital, Boston, MA 02115, USA
    Eur Respir J 32:938-44. 2008
    ..activator inhibitor type 1), member 2 (SERPINE2), glutathione S-transferase pi (GSTP1) and surfactant protein B (SFTPB) were genotyped in 389 non-Hispanic white participants in the National Emphysema Treatment Trial...
  2. 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...
  3. ncbi Structure and organization of the gene encoding human pulmonary surfactant proteolipid SP-B
    T J Pilot-Matias
    Corporate Molecular Biology, Abbott Laboratories, IL 60064
    DNA 8:75-86. 1989
    ..Southern blotting of human genomic DNA probed with SP-B cDNA indicated the presence of only one SP-B gene in the human genome, and the gene was localized to chromosome 2...
  4. pmc A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds
    L M Nogee
    Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287
    J Clin Invest 93:1860-3. 1994
    ..We conclude that this mutation is responsible for SP-B deficiency and neonatal alveolar proteinosis in multiple families and speculate that the disorder is more common than was recognized previously...
  5. ncbi Intracellular localization of processing events in human surfactant protein B biosynthesis
    A Korimilli
    Division of Neonatology, University of Pennsylvania School of Medicine, Department of Pediatrics, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 275:8672-9. 2000
    ....
  6. pmc Differences in N-linked glycosylation between human surfactant protein-B variants of the C or T allele at the single-nucleotide polymorphism at position 1580: implications for disease
    Guirong Wang
    Department of Cellular and Molecular Physiology, The Pennsylvania State University, College of Medicine, Hershey, PA 17033, USA
    Biochem J 369:179-84. 2003
    ..Further, we speculate that, given the fact that this SNP is found frequently in the general population, N-linked glycosylation at residue Asn129 interferes with SP-B processing, secretion and folding under certain disease conditions...
  7. ncbi Nitric oxide inhibits surfactant protein B gene expression in lung epithelial cells
    Darrell Salinas
    Department of Molecular Biology, University of Texas Health Center at Tyler, Tyler, Texas 75708 3154, USA
    Am J Physiol Lung Cell Mol Physiol 285:L1153-65. 2003
    ..These data imply that reduced SP-B expression due to elevated NO levels can contribute to lung injury...
  8. ncbi Surfactant protein B in type II pneumocytes and intra-alveolar surfactant forms of human lungs
    Frank Brasch
    Department of Anatomy, University of Gottingen, Gottingen, Germany
    Am J Respir Cell Mol Biol 30:449-58. 2004
    ..In human lungs, mature SP-B is involved in the structural organization of lamellar bodies and tubular myelin by the formation of core particles...
  9. ncbi Involvement of napsin A in the C- and N-terminal processing of surfactant protein B in type-II pneumocytes of the human lung
    Frank Brasch
    Department of Anatomy, University of Gottingen, Kreuzbergring 36, D 37075 Gottingen, Germany
    J Biol Chem 278:49006-14. 2003
    ..Two of the four processing products generated in vitro were also detected in type-II pneumocytes. In conclusion, our results show that napsin A is involved in the N- and C-terminal processing of proSP-B in type-II pneumocytes...
  10. ncbi Dysfunction of pulmonary surfactant in chronically ventilated premature infants
    Jeffrey D Merrill
    Neonatology, Department of Pediatrics, University of Pennsylvania School of Medicine, USA
    Pediatr Res 56:918-26. 2004
    ..We conclude that most premature infants requiring continued respiratory support after 7 d of age experience transient episodes of dysfunctional surfactant that are associated with a deficiency of SP-B and SP-C...
  11. ncbi Surfactant-associated protein B kinetics in vivo in newborn infants by stable isotopes
    Paola Cogo
    Department of Pediatrics, University of Padova, Padova, and Pharmacy, Salesi Children Hospital, Ancona, Italy
    Pediatr Res 57:519-22. 2005
    ..SP-B half-life seemed to be shorter than DSPC half-life. These results agree with existing animal data. We conclude that the measurement of SP-B kinetics is feasible in vivo in humans by stable isotope technology...
  12. pmc Attempted replication of reported chronic obstructive pulmonary disease candidate gene associations
    Craig P Hersh
    Channing Laboratory, Pulmonary and Critical Care Division, and Hematology Oncology Division, Department of Medicine, Brigham and Women s Hospital, Boston, MA 02115, USA
    Am J Respir Cell Mol Biol 33:71-8. 2005
    ..02), a coding variant in surfactant protein B (SFTPB Thr131Ile) (P = 0.03), and the (GT)(31) allele of the heme oxygenase (HMOX1) promoter short tandem repeat (P = 0...
  13. pmc Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease
    Matthias Griese
    Kinderklinik and Poliklinik, Dr von Haunersches Kinderspital, Ludwig Maximilians University, Munich, Germany
    Respir Res 6:80. 2005
    ..The profile of these proteins in the alveolar space is unknown in such subjects...
  14. ncbi Genetic variant characterization in intron 4 of the surfactant protein B gene
    Aaron Hamvas
    Division of Newborn Medicine, Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine and St Louis Children s Hospital, St Louis, Missouri, USA
    Hum Mutat 26:494-5. 2005
    Genetic variants in intron 4 of the surfactant protein B gene SFTPB have been associated with pulmonary morbidity in newborn infants and adults...
  15. pmc Genetic association analysis of functional impairment in chronic obstructive pulmonary disease
    Craig P Hersh
    Channing Laboratory, Brigham and Women s Hospital, 181 Longwood Avenue, Boston, MA 02115, USA
    Am J Respir Crit Care Med 173:977-84. 2006
    ..This variation may reflect different COPD subtypes, which may have different genetic predispositions...
  16. pmc Comprehensive genetic variant discovery in the surfactant protein B gene
    Aaron Hamvas
    Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine and St Louis Children s Hospital, St Louis, Missouri 63110, USA
    Pediatr Res 62:170-5. 2007
    Completely penetrant mutations in the surfactant protein B gene (SFTPB) and >75% reduction of SFTPB expression disrupt pulmonary surfactant function and cause neonatal respiratory distress syndrome...
  17. pmc Use of human surfactant low molecular weight apoproteins in the reconstitution of surfactant biologic activity
    S D Revak
    Department of Immunology, Research Institute of Scripps Clinic, La Jolla, California 92037
    J Clin Invest 81:826-33. 1988
    ..5% SP 9 yielded a moderate increase. These data show the ability to produce biologically active surfactant by the addition of isolated LMW apoproteins to defined PL...
  18. ncbi Isolation of a cDNA clone encoding a high molecular weight precursor to a 6-kDa pulmonary surfactant-associated protein
    K A Jacobs
    J Biol Chem 262:9808-11. 1987
    ..Immunoprecipitation of the 42-kDa primary translation product is inhibited by the presence of the bovine 6-kDa protein. These observations suggest a precursor-product relationship of the 42-kDa protein to one of the 6-kDa proteins...
  19. ncbi Mapping the human pulmonary surfactant-associated protein B gene (SFTP3) to chromosome 2p12-->p11.2
    N C Vamvakopoulos
    Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda MD
    Cytogenet Cell Genet 68:8-10. 1995
    ..We have mapped the human SP-B gene (SFTP3) to chromosome 2, band 2p12-->p11.2 by fluorescent in situ hybridization.
  20. 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...
  21. ncbi Compound SFTPB 1549C-->GAA (121ins2) and 457delC heterozygosity in severe congenital lung disease and surfactant protein B (SP-B) deficiency
    M Tredano
    Service de Biochimie et Biologie Moléculaire, Hopital d Enfants Armand Trousseau, Paris, France
    Hum Mutat 14:502-9. 1999
    ..Homozygosity for a common mutation (1549C-->GAA, or 121ins2) of the SP-B-encoding gene (SFTPB) results in rapidly fatal respiratory failure, with complete absence of the mRNA and protein observed in lung ..
  22. ncbi N-terminally extended surfactant protein (SP) C isolated from SP-B-deficient children has reduced surface activity and inhibited lipopolysaccharide binding
    Jing Li
    Department of Medical Biochemistry and Biophysics, Karolinska Institutet, S 171 77 Stockholm, Sweden
    Biochemistry 43:3891-8. 2004
    ..The results of this study indicate that the early postnatal fatal respiratory distress seen in SP-B-deficient children is combined with the near absence of active variants of SP-C...
  23. 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 ..
  24. pmc Inherited surfactant deficiency caused by uniparental disomy of rare mutations in the surfactant protein-B and ATP binding cassette, subfamily a, member 3 genes
    Aaron Hamvas
    Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine and St Louis Children s Hospital, St Louis, MO 63110, USA
    J Pediatr 155:854-859.e1. 2009
    To characterize inheritance of homozygous, rare, recessive loss-of-function mutations in surfactant protein-B (SFTPB) or ATP binding cassette, subfamily A, member 3 (ABCA3) genes in newborns with lethal respiratory failure.
  25. ncbi Role of endogenous TGF-beta in glucocorticoid-induced lung type II cell differentiation
    Theresa M McDevitt
    Division of Neonatology, Department of Pediatrics, University of California San Francisco, 3333 California St, Suite 150, San Francisco, CA 94118 1981, USA
    Am J Physiol Lung Cell Mol Physiol 292:L249-57. 2007
    ..blockade of TGF-beta reduced serpine1, a TGF-beta-inducible gene, and increased gene expression for sftpa, sftpb, sftpc, and titf1, mimicking the response to hormone treatment...
  26. ncbi Relation between perceived stimulus intensity and exteroceptive reflex responses in the human masseter muscles
    P O Hansen
    Danish Pain Research Centre and Department of Neurology, Aarhus University Hospital
    Clin Neurophysiol 110:1290-6. 1999
    ..In the present study, the relationship between nociception and ES1, ISP and PSP was investigated...
  27. pmc C/EBP{alpha} is required for pulmonary cytoprotection during hyperoxia
    Yan Xu
    Cincinnati Children s Hospital Medical Center, University of Cincinnati, Ohio 45229 3039, USA
    Am J Physiol Lung Cell Mol Physiol 297:L286-98. 2009
    ..of number of genes regulating surfactant lipid and protein homeostasis, including Srebf, Scap, Lpcat1, Abca3, Sftpb, and Napsa...
  28. 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...
  29. ncbi Immunohistochemical markers in diagnosis of papillary thyroid carcinoma: Utility of HBME1 combined with CK19 immunostaining
    Michel R Nasr
    Department of Pathology, State University of New York Upstate Medical University, Syracuse, NY 13210, USA
    Mod Pathol 19:1631-7. 2006
    ..cases of papillary thyroid carcinoma were initially stained for HBME1, CK19, fibronectin1, Ki-67, Calretinin, p16, SFTPB and CITED1...
  30. doi Porcine lung surfactant protein B gene (SFTPB): cDNA sequencing, chromosomal location, and expression studies
    S Cirera
    Department of Animal and Veterinary Basic Sciences, Division of Genetics and Bioinformatics, Faculty of Life Sciences, University of Copenhagen, Copenhagen, Denmark
    Anim Biotechnol 19:127-37. 2008
    The porcine surfactant protein B (SFTPB) is a single copy gene on chromosome 3. Three different cDNAs for the SFTPB have been isolated and sequenced...
  31. doi Novel strategy to identify genetic risk factors for COPD severity: a genetic isolate
    C C van Diemen
    Dept of Epidemiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
    Eur Respir J 35:768-75. 2010
    ..MMP9, MMP12, tissue inhibitor of metalloprotease-1 gene (TIMP1), surfactant protein A1 gene (SFTPA1 ), SFTPA2, SFTPB, SFTPD, glutathione S-transferase P1 gene (GSTP1), and haem oxygenase 1 gene ( HMOX1)...
  32. ncbi A murine respiratory-inducing niche displays variable efficiency across human and mouse embryonic stem cell species
    Mark Denham
    Centre for Reproduction and Development, Monash Institute of Medical Research, Monash University, Clayton, Australia, Australia
    Am J Physiol Lung Cell Mol Physiol 292:L1241-7. 2007
    ..Expression of other terminal lung secretory unit markers (TITF1, SFTPA, and SFTPB) was not detected at any time point analyzed...
  33. pmc Calcineurin/Nfat signaling is required for perinatal lung maturation and function
    Vrushank Dave
    Section of Neonatology, Perinatal and Pulmonary Biology, Cincinnati Children s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
    J Clin Invest 116:2597-609. 2006
    ..3 (Nfatc3), a transcription factor modulated by calcineurin, was identified as a direct activator of Sftpa, Sftpb, Sftpc, Abca3, Foxa1, and Foxa2 genes...
  34. ncbi Evaluation and management of inherited disorders of surfactant metabolism
    Aaron Hamvas
    Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine and St Louis Children s Hospital, St Louis, Missouri 63110, USA
    Chin Med J (Engl) 123:2943-7. 2010
    ..outcomes of children with inherited disorders of surfactant metabolism due to mutations in the genes encoding surfactant proteins-B or -C (SFTPB, SFTPC), ATP binding cassette member A3 (ABCA3), and thyroid transcription factor (NKX2.1).
  35. ncbi Chromosomal localization of three pulmonary surfactant protein genes in the mouse
    K J Moore
    ABL Basic Research Program, NCI Frederick Cancer Research and Development Center, Maryland 21702
    Genomics 12:388-93. 1992
    ..The mouse map locations determined in this study for the Sftp genes are consistent with the locations of these genes on the human genetic map and the syntenic relationships between the human and the mouse genomes...
  36. ncbi Prenatal diagnosis of congenital alveolar proteinosis (surfactant protein B deficiency)
    M Stuhrmann
    Institut fur Humangenetik, Medizinische Hochschule, Hannover, Germany
    Prenat Diagn 18:953-5. 1998
    ..and both parents were heterozygous for a frame-shift mutation in codon 121 of the surfactant protein B gene (SFTP3-gene). Chorionic villus sampling (CVS) was performed in two subsequent pregnancies...
  37. ncbi Congenital alveolar proteinosis caused by a novel mutation of the surfactant protein B gene and misalignment of lung vessels in consanguineous kindred infants
    M Wallot
    Universitäts Klinikum Essen, Klinik fur Kinder und Jugendmedizin, Germany
    Eur J Pediatr 158:513-8. 1999
    ..One of them, a single base deletion, shifts the reading frame at amino acid 122 and creates a premature termination of translation in exon 6. No mature SP-B protein is produced...
  38. pmc Genotyping patients with recent blood transfusions
    Michelle Ng Gong
    Pulmonary and Critical Care Unit, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
    Epidemiology 14:744-7. 2003
    ..To investigate this possibility, we assayed DNA extracted from the blood and buccal cells of ICU patients for 2 common polymorphisms in the TNF-beta gene and the surfactant protein-B (SP-B) gene...
  39. ncbi PARP-2 interacts with TTF-1 and regulates expression of surfactant protein-B
    Yutaka Maeda
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    J Biol Chem 281:9600-6. 2006
    ..Both PARP-1 and PARP-2 enhanced the activity of the promoter of surfactant protein-B (Sftpb gene) but not other surfactant proteins in vitro...
  40. ncbi Novel mutations in the gene encoding ATP binding cassette protein member A3 (ABCA3) resulting in fatal neonatal lung disease
    Ola D Saugstad
    Department of Pediatric Research, Rikshospitalet Radiumhospitalet Health Trust, University of Oslo, Oslo, Norway
    Acta Paediatr 96:185-90. 2007
    ..To investigate whether intractable respiratory distress syndrome in three Norwegian term infants was due to mutations in the ABCA3 gene...
  41. pmc IL10 polymorphisms are associated with airflow obstruction in severe alpha1-antitrypsin deficiency
    Dawn L DeMeo
    Brigham and Women s Hospital, Channing Laboratory, 181 Longwood Avenue, Boston, MA 02115, USA
    Am J Respir Cell Mol Biol 38:114-20. 2008
    ..genes previously associated with asthma and/or COPD, including IL10, TNF, GSTP1, NOS1, NOS3, SERPINA3, SERPINE2, SFTPB, TGFB1, and EPHX1. All analyses were performed in a cohort of 378 PI ZZ individuals from 167 families...
  42. pmc Population and disease-based prevalence of the common mutations associated with surfactant deficiency
    Tami H Garmany
    Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Pediatr Res 63:645-9. 2008
    ..E292V was over-represented in newborns with RDS suggesting that E292V or its unique haplotype impart increased genetic risk for RDS...
  43. pmc Misexpression of MIA disrupts lung morphogenesis and causes neonatal death
    Sui Lin
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
    Dev Biol 316:441-55. 2008
    ..with exogenous MIA caused significant reductions in the expression of the lung differentiation markers Sftpa, Sftpb, Sftpc, and Abca3. Bitransgenic mice expressing MIA under the control of the SFTPC promoter after E16...
  44. pmc Recombination as a mechanism for sporadic mutation in the surfactant protein-C gene
    Amy D McBee
    Division of Newborn Medicine, Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri, USA
    Pediatr Pulmonol 43:443-50. 2008
    To determine haplotype background of common mutations in the genes encoding surfactant proteins B and C (SFTPB and SFTPC) and to assess recombination in SFTPC.
  45. pmc Surfactant proteins SP-B and SP-C and their precursors in bronchoalveolar lavages from children with acute and chronic inflammatory airway disease
    Oliver Tafel
    Lung Research Group, Children s Hospital of Ludwig Maximilian University, Munich, Germany
    BMC Pulm Med 8:6. 2008
    ..Their involvement and down-regulation in inflammatory processes has recently been proposed, but their level during neutrophilic human airway diseases are not yet known...
  46. pmc Developmental and genetic regulation of human surfactant protein B in vivo
    Aaron Hamvas
    Division of Newborn Medicine, the Edward Mallinckrodt Department of Pediatrics and St Louis Children s Hospital, St Louis, MO 63110, USA
    Neonatology 95:117-24. 2009
    ..Genetic and developmental disruption of surfactant protein B (SP-B) expression causes neonatal respiratory distress syndrome (RDS)...
  47. ncbi Recombinant production of a hybrid plasminogen activator composed of surfactant protein B and low-molecular-weight urokinase
    Clemens Ruppert
    Department of Internal Medicine, University of Giessen Lung Center, Giessen, Germany
    Thromb Haemost 100:1185-92. 2008
    ..We conclude that recombinant production of a fusion protein consisting of mature SP-B and uPA is feasible, when the SP-B moiety is fused to the C-terminus of urokinase...
  48. pmc Altered surfactant homeostasis and recurrent respiratory failure secondary to TTF-1 nuclear targeting defect
    Donatella Peca
    Department of Medical and Surgical Neonatology, Bambino Gesu Children s Hospital IRCCS, Rome, Italy
    Respir Res 12:115. 2011
    Mutations of genes affecting surfactant homeostasis, such as SFTPB, SFTPC and ABCA3, lead to diffuse lung disease in neonates and children. Haploinsufficiency of NKX2...
  49. pmc Synergistic effect of caffeine and glucocorticoids on expression of surfactant protein B (SP-B) mRNA
    Markus Fehrholz
    Children s Hospital, University of Wuerzburg, Wuerzburg, Germany
    PLoS ONE 7:e51575. 2012
    ..Co-administration of caffeine and corticosteroids may therefore be of benefit in surfactant homeostasis...
  50. doi Detection of surfactant proteins A, B, C, and D in human nasal mucosa and their regulation in chronic rhinosinusitis with polyps
    Martin Schicht
    Department of Anatomy II, University of Erlangen Nurnberg, Germany
    Am J Rhinol Allergy 27:24-9. 2013
    ..The objective of this study was to investigate the protein expression of all four SPs. A specific aim was detection and characterization of SP-C, which had previously not been confirmed in human nasal mucosa...
  51. pmc LPS-treated macrophage cytokines repress surfactant protein-B in lung epithelial cells
    Kiflai Bein
    Department of Environmental and Occupational Health, Graduate School of Public Health, University of Pittsburgh, Pittsburgh, PA 15219 3130, USA
    Am J Respir Cell Mol Biol 49:306-15. 2013
    In the mouse lung, Escherichia coli LPS can decrease surfactant protein-B (SFTPB) mRNA and protein concentrations...
  52. doi Glucagon-like peptide-1 (GLP-1) reduces mortality and improves lung function in a model of experimental obstructive lung disease in female mice
    Niels Erik Viby
    PhD, MD, Department of Biomedical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, Blegdamsvej 3, DK 2200 Copenhagen N, Denmark
    Endocrinology 154:4503-11. 2013
    ..001). Survival proportions were significantly increased in GLP-1R agonist-treated mice (P < .01). SFTPB and SFTPA were down-regulated and the expression of inflammatory cytokines were increased in mice with obstructive ..
  53. ncbi Luciferase assay to screen tumour-specific promoters in lung cancer
    Rong Xu
    Institute of Molecular Medicine, Huaqiao University, Quanzhou, China E mail
    Asian Pac J Cancer Prev 14:6557-62. 2014
    ..Specific promoters could improve efficiency and ensure the safety of gene therapy. The aim of our study was to screen examples for lung cancer...
  54. doi Association of surfactant protein B gene with chronic obstructive pulmonary disease susceptibility
    J Yang
    Department of Respiratory Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
    Int J Tuberc Lung Dis 18:1378-84. 2014
    ..Several candidate genes have been proposed for COPD, including surfactant protein B (SFTPB)...
  55. pmc Surfactant protein B gene polymorphisms is associated with risk of bronchopulmonary dysplasia in Chinese Han population
    Sheng Zhang
    Affiliated Bayi Children s Hospital, General Military Hospital of Beijing PLA Beijing, China
    Int J Clin Exp Pathol 8:2971-8. 2015
    ..To investigate the relationship between Surfactant protein B (SP-B) gene polymorphisms and bronchopulmonary dysplasia (BPD) development in preterm infants of China Han ethnic population...
  56. doi High-throughput alternative splicing detection using dually constrained correspondence analysis (DCCA)
    Florent Baty
    Department of Pulmonary Medicine, Cantonal Hospital St Gallen, Switzerland Electronic address
    J Biomed Inform 58:175-85. 2015
    ..Splicing candidates reveal a series of genes related to carcinogenesis (SFTPB), cell adhesion (STAB2, PCDH15, HABP2), tumor aggressiveness (ARNTL2), apoptosis, proliferation and ..
  57. doi Population-based frequency of surfactant dysfunction mutations in a native Chinese cohort
    Yu Jun Chen
    Division of Neonatology, Department of Pediatrics, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
    World J Pediatr 12:190-5. 2016
    ..Rare mutations in surfactant-associated genes contribute to neonatal respiratory distress syndrome. The frequency of mutations in these genes in the Chinese population is unknown...
  58. pmc DIFFERENTIAL SUSCEPTIBILITY OF HUMAN SP-B GENETIC VARIANTS ON LUNG INJURY CAUSED BY BACTERIAL PNEUMONIA AND THE EFFECT OF A CHEMICALLY MODIFIED CURCUMIN
    Yongan Xu
    Department of Surgery, SUNY Upstate Medical University, Syracuse, New York Department of Emergency Medicine, Second Affiliated Hospital Zhejiang University school of Medicine, Hangzhou, People s Republic of China Department of Biochemistry and Molecular Biology, Tianjin Medical University, Tianjin, People s Republic of China Department of Pharmacological Sciences Department of Oral Biology and Pathology, State University of New York at Stony Brook, Stony Brook, New York
    Shock 45:375-84. 2016
    ..05), and MMPs-2, -9, -12 activities (P < 0.05). We conclude that mice with SP-B-C allele are more susceptible to S. aureus pneumonia than mice with SP-B-T allele, and that CMC2.24 attenuates lung injury thus reducing mortality...
  59. pmc Interindividual variability in the expression of surfactant protein A and B in the human lung during development
    F Cau
    Hospital Nostra Signora di Bonaria
    Eur J Histochem 60:2678. 2016
    ....
  60. doi Insertions and Deletions Target Lineage-Defining Genes in Human Cancers
    Marcin Imielinski
    Department of Pathology and Laboratory Medicine, Englander Institute for Precision Medicine, Institute for Computational Biomedicine, and Meyer Cancer Center, Weill Cornell Medicine, New York, NY 10065, USA New York Genome Center, New York, NY 10013, USA Electronic address
    Cell . 2017
    ..noncoding somatic mutational hotspots near VMP1/MIR21 and indel hotspots in surfactant protein genes (SFTPA1, SFTPB, and SFTPC)...
  61. ncbi Transforming growth factor-beta inhibits pulmonary surfactant protein B gene transcription through SMAD3 interactions with NKX2.1 and HNF-3 transcription factors
    Changgong Li
    Department of Pediatrics, Women s and Children s Hospital, University of Southern California School of Medicine, Los Angeles, California 90033, USA
    J Biol Chem 277:38399-408. 2002
    ..1 was not affected. We conclude that SMAD3 interactions with the positive regulators NKX2.1 and HNF-3 underlie the molecular basis for TGF-beta-induced repression of Sp-B gene transcription...
  62. ncbi Dimeric surfactant protein B peptide sp-b(1-25) in neonatal and acute respiratory distress syndrome
    Frans J Walther
    Research and Education Institute at Harbor UCLA Medical Center, Torrance, California 90502, USA
    Exp Lung Res 28:623-40. 2002
    ..The data suggest that dSP-B(1-25) is more efficient in restoring lung function in neonatal RDS and ARDS than mSP-B(1-25) surfactant...
  63. pmc A combination of molecular markers accurately detects lymph node metastasis in non-small cell lung cancer patients
    Liqiang Xi
    Department of Surgery, University of Pittsburgh, Pittsburgh, Pennsylvania, USA
    Clin Cancer Res 12:2484-91. 2006
    ..A combination of three markers (SFTPB, TACSTD1, and PVA) was identified that provided perfect classification of benign and positive nodes in all sample ..
  64. ncbi Surfactant protein B gene polymorphism in preterm babies with respiratory distress syndrome
    P P R Lyra
    Departamento de Pediatria, Universidade de Sao Paulo, SP, Brasil
    Braz J Med Biol Res 44:66-72. 2011
    ..1681; 95%CI = 0.0426-0.6629). We did not detect differences in the frequencies of the other polymorphisms between the two groups of newborns...
  65. doi Kinetics of plasma SPB and RAGE during mechanical ventilation in patients undergoing major vascular surgery
    Piergiuseppe Agostoni
    Centro Cardiologico Monzino, IRCCS, Milan, Italy
    Respir Physiol Neurobiol 178:256-60. 2011
    ..SPB(N) increase (T(END)-T0), but not RAGE(N), was related to ΔPaO(2)/FiO2 changes during mechanical ventilation (r=0.575, p=0.01). Plasma RAGE(N) and SPB(N) kinetics in patients undergoing major vascular surgery are different...
  66. doi Identification of seven novel SMPD1 mutations causing Niemann-Pick disease types A and B
    P Irun
    Departamento de Bioquimica y Biologia Molecular y Celular, Universidad de Zaragoza, Zaragoza, Spain Instituto de Investigación Sanitaria de Aragón IIS, Zaragoza, Spain Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER, Zaragoza U752, Barcelona U719, Spain
    Clin Genet 84:356-61. 2013
    ..Sequencing the SMDP1 gene revealed eight previously described mutations and seven novel mutations including four missense [c...
  67. pmc Surfactant protein B deficiency and gene mutations for neonatal respiratory distress syndrome in China Han ethnic population
    Xiaojuan Yin
    Affiliated Bayi Children s Hospital, Beijing Military Region General Hospital Beijing, China
    Int J Clin Exp Pathol 6:267-72. 2013
    ..To determine whether the SP-B deficiency and gene mutations in exon 4 is associated with neonatal RDS in China Han ethnic population...
  68. doi Differential effects of insulin and dexamethasone on pulmonary surfactant-associated genes and proteins in A549 and H441 cells and lung tissue
    Zdenek Rucka
    Centre for Biomedical Image Analysis, Faculty of Informatics, Masaryk University, 60200 Brno, Czech Republic
    Int J Mol Med 32:211-8. 2013
    ..the expression and mRNA transcription of 4 pulmonary surfactant-associated proteins [surfactant protein (SFTP)A, SFTPB, SFTPC and SFTPD] were examined...
  69. pmc ErbB4 is an upstream regulator of TTF-1 fetal mouse lung type II cell development in vitro
    Katja Zscheppang
    Department of Pediatrics, Hannover Medical School, Hannover 30625, Germany Division of Newborn Medicine, Floating Hospital for Children at Tufts Medical Center, Boston, MA 02111, USA
    Biochim Biophys Acta 1833:2690-702. 2013
    ..We speculate that ErbB4 is an upstream regulator of TTF-1 and regulates Sftpb expression via this pathway in alveolar type II cells...
  70. pmc cDNA and deduced amino acid sequence of human pulmonary surfactant-associated proteolipid SPL(Phe)
    S W Glasser
    Proc Natl Acad Sci U S A 84:4007-11. 1987
    ..These proteins, and specifically SPL(Phe), may therefore be useful for synthesis of replacement surfactants for treatment of hyaline membrane disease in newborn infants or of other surfactant-deficient states...
  71. 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...
  72. ncbi Clinical biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism
    M Tredano
    Service de Biochimie et Biologie Moléculaire, H pital d Enfants Armand Trousseau, Paris, France
    Clin Chem Lab Med 39:90-108. 2001
    ..material is stored within the alveoli, and which represents an autosomal recessive Mendelian entity linked to the SFTPB gene (MIM 1786640)...
  73. pmc Surfactant protein B gene variations enhance susceptibility to squamous cell carcinoma of the lung in German patients
    C Seifart
    Department of Internal Medicine, Division of Respiratory and Critical Care Medicine, Philipps University of Marburg, Baldingerstrasse, 35043 Marburg, Germany
    Br J Cancer 87:212-7. 2002
    ..These results indicate an association of the surfactant protein B intron 4 variants and/or its flanking loci with mechanisms that may enhance lung cancer susceptibility, especially to squamous cell carcinoma of the lung...
  74. ncbi Polymorphisms of surfactant protein B encoding gene: modifiers of the course of neonatal respiratory distress syndrome?
    Vassiliki Makri
    Department of Paediatrics and Neonatology, University Children s Hospital, Justus Liebig University, Feulgenstrasse 12, 35392 Giessen, Germany
    Eur J Pediatr 161:604-8. 2002
    ..05) and the need for surfactant administration were also higher in group 2 than in group 1 (43.2% versus 72.4%, P < 0.01). Duration of mechanical ventilation and rate of chronic lung disease at 36 weeks were comparable in both groups...
  75. ncbi Surfactant protein B polymorphism and respiratory distress syndrome in premature twins
    Riitta Marttila
    Department of Paediatrics and Biocenter Oulu, University of Oulu, Finland
    Hum Genet 112:18-23. 2003
    ..The traditional twin concordance study is insufficient to evaluate genetic predisposition to RDS or other diseases that are confounded by the birth order or multiple pregnancy in itself...
  76. 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...
  77. ncbi Polymorphism in the surfactant protein-B gene, gender, and the risk of direct pulmonary injury and ARDS
    Michelle Ng Gong
    Pulmonary and Critical Care Unit, Massachusetts General Hospital, Harvard Medical School, Goston, MA 02115, USA
    Chest 125:203-11. 2004
    ..To investigate the possibility of variable genetic susceptibility to ARDS, we examined the association between ARDS and a polymorphism in intron 4 of the surfactant protein-B (SP-B) gene...
  78. ncbi [Biologic significance of thyroid transcription factor-1 and surfactant protein expression in pulmonary sclerosing hemangioma]
    Xiang Hua Yi
    Department of Pathology, Shanghai Pulmonary Hospital, Shanghai 200433, China
    Zhonghua Jie He He Hu Xi Za Zhi 26:776-80. 2003
    ..To investigate the biologic significance of thyroid transcription factor-1 (TTF-1) and surfactant protein A (SP-A) and SP-B expression in pulmonary sclerosing hemangioma (PSH)...
  79. ncbi Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease
    Mohammed Tredano
    Service de Biochimie et Biologie Moléculaire, hôpital d enfants Armand Trousseau AP HP, Paris, France
    Am J Med Genet A 126:18-26. 2004
    ..cases with unexplained respiratory distress (URD) in which surfactant protein B (SP-B) deficiency related to SFTPB mutation had been ruled out. One patient with complete SP-C deficiency had no detectable mutation of SFTPC...
  80. ncbi Data mining and multiparameter analysis of lung surfactant protein genes in bronchopulmonary dysplasia
    Meri Rova
    Department of Paediatrics and Biocenter Oulu, University of Oulu, Oulu, Finland
    Hum Mol Genet 13:1095-104. 2004
    ....
  81. ncbi Mapping and analysis of the lytic and fusogenic domains of surfactant protein B
    Marnie A Ryan
    Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
    Biochemistry 44:861-72. 2005
    ....
  82. ncbi Surfactant protein B gene variations and susceptibility to lung cancer in chromate workers
    Ashraf A Ewis
    Single Molecule Bioanalysis Laboratory, National Institute of Advanced Industrial Science and Technology AIST, Takamatsu, Japan
    Am J Ind Med 49:367-73. 2006
    ....
  83. 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...
  84. ncbi Stage-specific effects of cAMP signaling during distal lung epithelial development
    Jingsong Xu
    Division of Pulmonary and Critical Care Medicine, Brigham and Women s Hospital at Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA
    J Biol Chem 281:38894-904. 2006
    ..Second, there are cAMP-responsive elements in the heterologous promoters of Sftpb and Sftpa genes...
  85. ncbi The C/A(-18) polymorphism in the surfactant protein B gene influences transcription and protein levels of surfactant protein B
    Wendy K Steagall
    Pulmonary Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bldg 10, Rm 6D03, MSC 1590, Bethesda, MD 20892 1590, USA
    Am J Physiol Lung Cell Mol Physiol 292:L448-53. 2007
    ..Differences in amounts of SP-B resulting from the promoter SNP could affect the clinical presentation of pulmonary disease...
  86. ncbi Expression and localization of surfactant proteins in human nasal epithelium
    Jin Kook Kim
    Dept of Otorhinolaryngology Head and Neck Sugery, Konkuk University College of Medicine, Seoul
    Am J Physiol Lung Cell Mol Physiol 292:L879-84. 2007
    ..We demonstrate that SP-A, SP-B, and SP-D are expressed in human nasal mucosa and cultured NHNE cells. Further study of the functional role of SPs in the upper airway is required...
  87. ncbi A major deletion in the surfactant protein-B gene causing lethal respiratory distress
    Daniel J Wegner
    Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine and St Louis Children s Hospital, MO 63110, USA
    Acta Paediatr 96:516-20. 2007
    Loss of function mutations in the surfactant protein-B gene (SFTPB) cause lethal neonatal respiratory distress due to reduced or absent expression of mature surfactant protein B (SP-B, encoded in exons 6 and 7)...
  88. ncbi A 10-gene classifier for distinguishing head and neck squamous cell carcinoma and lung squamous cell carcinoma
    Anil Vachani
    University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, USA
    Clin Cancer Res 13:2905-15. 2007
    ..The distinction between a lung metastasis and a primary LSCC is currently based on certain clinical and histologic criteria, although the accuracy of this approach remains in question...
  89. ncbi Comparison of surfactant protein B polymorphisms of healthy term newborns with preterm newborns having respiratory distress syndrome
    P P R Lyra
    Departamento de Pediatria, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brasil
    Braz J Med Biol Res 40:779-86. 2007
    ..These data demonstrate an association of GG genotype with RDS...
  90. pmc Pepsinogen C proteolytic processing of surfactant protein B
    Kristin D Gerson
    Division of Neonatology, Department of Pediatrics, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 283:10330-8. 2008
    ..Inhibition of SP-B production recapitulated the SP-B-deficient phenotype evident by aberrant lamellar body genesis. Together, these data support a primary role for pepsinogen C in SP-B proteolytic processing in alveolar type 2 cells...
  91. ncbi Surfactant protein B deficiency caused by a novel mutation involving multiple exons of the SP-B gene
    F A B A Schuerman
    Department of Neonatology, Academic Medical Center, Amsterdam, The Netherlands
    Eur J Med Res 13:281-6. 2008
    Inability to produce surfactant protein (SP)-B causes fatal neonatal respiratory disease. Homozygosity for a frameshift mutation (121ins2) in the gene encoding SP-B (SFTPB) is the predominant but not the exclusive cause of disease.
  92. pmc Genetic associations with hypoxemia and pulmonary arterial pressure in COPD
    Peter J Castaldi
    Channing Laboratory, Brigham and Women s Hospital, 181 Longwood Ave, Boston, MA 02115, USA
    Chest 135:737-44. 2009
    ..We sought to identify genetic polymorphisms associated with these traits in a population of patients with severe COPD from the National Emphysema Treatment Trial (NETT)...
  93. pmc Forkhead Box m1 transcription factor is required for perinatal lung function
    Tanya V Kalin
    Division of Pulmonary Biology, Cincinnati Children s Hospital Research Foundation, 3333 Burnet Avenue, Cincinnati, OH 45229, USA
    Proc Natl Acad Sci U S A 105:19330-5. 2008
    ..Foxm1 directly bound and induced transcriptional activity of the mouse surfactant protein B and A (Sftpb and Sftpa) promoters in vitro, indicating that Foxm1 is a direct transcriptional activator of these genes...
  94. pmc Genetic association analysis of COPD candidate genes with bronchodilator responsiveness
    Woo Jin Kim
    Channing Laboratory, Brigham and Women s Hospital, Boston, MA 02115, USA
    Respir Med 103:552-7. 2009
    ..Genotyping was completed for 122 single nucleotide polymorphisms (SNPs) in six candidate genes (EPHX1, SFTPB, TGFB1, SERPINE2, GSTP1, ADRB2)...
  95. pmc Genetic disorders of surfactant dysfunction
    Susan E Wert
    Perinatal Institute, Section of Neonatology, Perinatal and Pulmonary Biology, Cincinnati Children s Hospital Medical Center, and the Department of Pediatrics, University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
    Pediatr Dev Pathol 12:253-74. 2009
    ..In general, mutations in the SP-B gene SFTPB are associated with fatal respiratory distress in the neonatal period, and mutations in the SP-C gene SFTPC are ..
  96. pmc Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease
    Jeffrey A Whitsett
    Perinatal Institute, Section of Neonatology, Perinatal and Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    Annu Rev Med 61:105-19. 2010
    ..Mutations in the ABCA3, SFTPA, SFTPB, SFTPC, SCL34A2, and TERT genes disrupt type II cell function and/or surfactant homeostasis, causing neonatal ..
  97. doi Surfactant protein-B 121ins2 heterozygosity, reduced pulmonary function, and chronic obstructive pulmonary disease in smokers
    Marie Baekvad-Hansen
    Department of Clinical Biochemistry, Herlev Hospital, Copenhagen University Hospital, Denmark
    Am J Respir Crit Care Med 181:17-20. 2010
    ..Individuals heterozygous for this mutation have partial absence of surfactant protein-B and could be at risk of lung disease when exposed to additional risk factors for impaired surfactant function such as tobacco smoking...
  98. doi NKX2-1 mutations leading to surfactant protein promoter dysregulation cause interstitial lung disease in "Brain-Lung-Thyroid Syndrome"
    Loic Guillot
    INSERM UMR 938, UPMC, Universite Paris 6, France
    Hum Mutat 31:E1146-62. 2010
    NKX2-1 (NK2 homeobox 1) is a critical regulator of transcription for the surfactant protein (SP)-B and -C genes (SFTPB and SFTPC, respectively). We identified and functionally characterized two new de novo NKX2-1 mutations c.493C>T (p...
  99. pmc Association of COPD candidate genes with computed tomography emphysema and airway phenotypes in severe COPD
    W J Kim
    Channing Laboratory, Brigham and Women s Hospital, Boston, MA, 02115 USA
    Eur Respir J 37:39-43. 2011
    ..Further investigation will be required to replicate these genetic associations for emphysema and airway wall phenotypes...
  100. doi Accuracy of pregnancy specific protein-B test for early pregnancy diagnosis in dairy cattle
    Juan E Romano
    Department of Large Animal Clinical Sciences, College of Veterinary Medicine and Biomedical Sciences, Texas A and M University, College Station, TX 77843 4475, USA
    Theriogenology 74:932-9. 2010
    ..In conclusion, PSP-B ELISA was a sensitive, specific, and accurate test for pregnancy diagnosis (relative to TRUS) at Days 28, 30, and 35 after breeding...

Research Grants14

  1. OZONE EFFECTS ON FUNCTION OF SURFACTANT PROTEIN VARIANTS
    Joanna Floros; Fiscal Year: 2010
    ..Knowledge gained may help explain and perhaps link the in vitro data with the human genetic association data, and provide insight in the understanding of the value of the SP-A gene duplication. ..
  2. Growth Factor Protection in Acute Lung Injury
    GEORGE LEIKAUF; Fiscal Year: 2009
    ..and TGFp interactions that modulate critical gene expression, focusing on the cis-acting elements that control Sftpb promoter transactivation...
  3. Asthma Candidate Genes in Alpha 1-Antitrypsin Deficiency
    Dawn DeMeo; Fiscal Year: 2007
    ..The identification of modifying factors of COPD in AAT deficiency may have significant public health relevance to COPD patients without AAT deficiency. ..
  4. The Role of surfactant protein genetic variants in cyst*
    Joanna Floros; Fiscal Year: 2005
    ..aeruginosa and its clearance, and provide the basis for further consideration of novel therapeutic strategies to treat CF-related lung disease. ..
  5. In Vivo Metabolism of Pulmonary Surfactant in Infants
    Aaron Hamvas; Fiscal Year: 2005
    ..abstract_text> ..
  6. EPIDEMIOLOGY OF SURFACTANT PROTEIN-B DEFICIENCY
    FRANCIS SESSIONS COLE; Fiscal Year: 2013
    ..variants, allelic heterogeneity, low linkage disequilibrium in pulmonary surfactant metabolic network genes (SFTPB, SFTPC, and ABCA3), and natural selection against variants that disrupt neonatal lung function suggest that rare, ..
  7. SURFACTANT PATHWAY GENE VARIANTS AMONG INFANTS WITH RESPIRATORY DISTRESS SYNDROME
    JENNIFER WAMBACH; Fiscal Year: 2013
    ..mechanism is suggested by studies of twins, ethnicity, gender, and lethal mutations in surfactant-related genes (SFTPB, SFTPC, and ABCA3)...
  8. Nilufer Ertekin-Taner; Fiscal Year: 2016
    ..These molecular mechanisms will constitute modifiable drug targets, which will impact PSP and other related neurodegenerative diseases. ..
  9. SHH and Alveolar Formation
    Jeffrey Whitsett; Fiscal Year: 2006
    ..abstract_text> ..
  10. PERINATAL EXPRESSION OF SURFACTANT PROTEIN GENE SP-B
    Jeffrey Whitsett; Fiscal Year: 2007
    ..Transcriptional responses to decreased SP-B will be determined using RNA microarray, to identify the molecular pathways mediating or compensating for reduced SP-B/surfactant function in vivo. [unreadable] [unreadable]..
  11. Transcriptional Control of Respiratory Epithelial Progenitor Cells
    Jeffrey A Whitsett; Fiscal Year: 2010
    ..A Stem Cell Consortium will be established at CCHMC, Univ. of Cincinnati College of Medicine to develop education, training, and research bridging hematopoietic stem cell and pulmonary stem cell biology on our campus. ..
  12. 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. ..
  13. Host Genetic Factors in the HIV Virus Life-Cycle and HIV Disease Progression
    Jairam Lingappa; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable]..
  14. ABCA3 and Alveolar Homeostasis
    Jeffrey A Whitsett; Fiscal Year: 2010
    ....