Research Topics
Genomes and Genes | T E WeaverSummaryAffiliation: Children's Hospital Medical Center Country: USA Publications
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Detail Information
Publications
Antimicrobial activity of native and synthetic surfactant protein B peptidesMarnie 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...
Biogenesis of lamellar bodies, lysosome-related organelles involved in storage and secretion of pulmonary surfactantTimothy E Weaver
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
Semin Cell Dev Biol 13:263-70. 2002....
Function of surfactant proteins B and CT E Weaver
Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
Annu Rev Physiol 63:555-78. 2001..This review attempts to integrate recent findings in humans and transgenic mice with the results of in vitro studies to provide a better understanding of the functions of SP-B and SP-C and the structural basis for their actions...
Use of knockout mice to study surfactant protein structure and functionT 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...
Synthesis, processing and secretion of surfactant proteins B and CT E Weaver
Children s Hospital Medical Center, Division of Pulmonary Biology, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
Biochim Biophys Acta 1408:173-9. 1998..8000 and 4000, respectively. The biosynthetic pathway leading to generation and secretion of the biophysically active mature SP-B and SP-C peptides is reviewed...
Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn miceJ 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...
The role of homodimers in surfactant protein B function in vivoD C Beck
Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
J Biol Chem 275:3365-70. 2000..Taken together, these data indicate a role for SP-B dimer in surface tension reduction in the alveolus...
Interleukin-5-mediated allergic airway inflammation inhibits the human surfactant protein C promoter in transgenic miceA Mishra
Division of Pulmonary Medicine, Allergy and Clinical Immunology, Department of Pediatrics, Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
J Biol Chem 276:8453-9. 2001..These results suggest that alterations in surfactant protein levels may contribute to immune and airway dysfunction in asthma...
Secretion of surfactant protein C, an integral membrane protein, requires the N-terminal propeptideJ J Conkright
Division of Pulmonary Biology, Children's Hospital Medical Center, Cincinnati, Ohio 45229-3039, USA
J Biol Chem 276:14658-64. 2001..Collectively, these studies indicate that the N-terminal propeptide of SP-C is required for intracellular sorting and secretion of SP-C...
Monoclonal antibody to thyroid transcription factor-1: production, characterization, and usefulness in tumor diagnosisA Holzinger
Division of Pulmonary Biology, University of Cincinnati College of Medicine, Ohio 45229 3039, USA
Hybridoma 15:49-53. 1996..These findings demonstrate that anti-TTF-1 MAb 8G7G3/1 specifically binds TTF-1 in cell extracts and tissues and can be used to distinguish between lung and nonlung origin of a tumor...
Partial SP-B deficiency perturbs lung function and causes air space abnormalitiesLori L Nesslein
Cincinnati Children's Hospital Medical Center, Division of Neonatology, 3333 Burnet Ave, Cincinnati, OH 45229-3039, USA
Am J Physiol Lung Cell Mol Physiol 288:L1154-61. 2005..Chronic, nonuniform SP-B deficiency perturbed pulmonary function and caused air space enlargement...
Reversibility of lung inflammation caused by SP-B deficiencyMachiko Ikegami
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
Am J Physiol Lung Cell Mol Physiol 289:L962-70. 2005..SP-B reversed inflammation and maintained lung function in vivo, indicating its potential utility for the prevention and treatment of pulmonary injury and surfactant deficiency...
Critical structure-function determinants within the N-terminal region of pulmonary surfactant protein SP-BAlicia G Serrano
Departamento de Bioquimica y Biologia Molecular I, Facultad de Biologia, Universidad Complutense, Madrid, Spain
Biophys J 90:238-49. 2006....
N-terminally extended surfactant protein (SP) C isolated from SP-B-deficient children has reduced surface activity and inhibited lipopolysaccharide bindingJing 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...
Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-CJames P Bridges
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, The University of Cincinnati College of Medicine, Cincinnati, OH 45267, USA
J Cell Biol 172:395-407. 2006..The wide variability in the age of onset of ILD in patients with SFTPC mutations may be related to environmental insults that ultimately overwhelm the homeostatic cytoprotective response...
Surface tension influences cell shape and phagocytosis in alveolar macrophagesHiroko 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..SP-B controls surface tension by forming a surfactant phospholipid film that regulates shape and nonspecific phagocytic activity of alveolar macrophages on the alveolar surface...
The peptidoglycan-degrading property of lysozyme is not required for bactericidal activity in vivoJames A Nash
Division of Pulmonary Biology, Department of Pediatrics, Cincinnati Children s Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA
J Immunol 177:519-26. 2006..These results indicate that the muramidase activity of lysozyme is not required for bacterial killing in vitro or in vivo...
Processing of pulmonary surfactant protein B by napsin and cathepsin HTakayuki 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...
Mouse lysozyme M is important in pulmonary host defense against Klebsiella pneumoniae infectionPhilipp Markart
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
Am J Respir Crit Care Med 169:454-8. 2004..01). Deficiency of lysozyme M in the lungs increased susceptibility to K. pneumoniae infection, whereas increased expression of lysozyme conferred resistance to infection and enhanced survival...
Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic miceJames P Bridges
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
J Biol Chem 278:52739-46. 2003..1728 G --> A mutation causes misfolding of the SP-C proprotein with subsequent induction of the unfolded protein response and endoplasmic reticulum-associated degradation pathways ultimately resulting in disrupted lung morphogenesis...
Overexpression of surfactant protein-C mature peptide causes neonatal lethality in transgenic miceJuliana 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...
Deficiency of SP-B reveals protective role of SP-C during oxygen lung injuryMachiko Ikegami
Children s Hospital Medical Center, Division of Pulmonary Biology, Cincinnati, Ohio 45229 3039, USA
J Appl Physiol 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...
Maintenance of the mouse type II cell phenotype in vitroWard R Rice
Division of Pulmonary Biology, Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
Am J Physiol Lung Cell Mol Physiol 283:L256-64. 2002..2 +/- 2.3 mN/m. This is the first description of the synthesis and secretion of a functional surfactant complex by mouse type II cells after 7 days in primary culture...
Intersubunit disulfide bridge is not required for the protective role of SP-B against lung inflammationMachiko Ikegami
Division of Pulmonary Biology, Cincinnati Children s Hospital, Cincinnati, Ohio 45229 3039, USA
J Appl Physiol 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...
Hydrophobic surfactant proteins in lung function and diseaseJeffrey A Whitsett
Division of Neonatology, Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, OH 45229-3039, USA
N Engl J Med 347:2141-8. 2002
Surfactant protein B inhibits endotoxin-induced lung inflammationRalph 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....
SP-B deficiency causes respiratory failure in adult miceKristin 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...
TTF-1 phosphorylation is required for peripheral lung morphogenesis, perinatal survival, and tissue-specific gene expressionMario 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...
Use of transgenic mice to study lung morphogenesis and functionJames P Bridges
Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA
ILAR J 47:22-31. 2006..In this review, selected transgenic mouse models are highlighted to illustrate the power of this technology, which in many cases has provided important insights that otherwise could not have been obtained...
Mapping and analysis of the lytic and fusogenic domains of surfactant protein BMarnie 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....
The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segmentHanna Johansson
Department of Molecular Biosciences, Swedish University of Agricultural Sciences, The Biomedical Centre, Uppsala, Sweden
J Biol Chem 281:21032-9. 2006..These findings suggest that CTproSP-C binds nonhelical SP-C and thereby prevents beta-sheet aggregation and that mutations in CTproSP-C can interfere with this function...
Comparison of the microbicidal and muramidase activities of mouse lysozyme M and PPhilipp Markart
Zentrum fur Innere Medizin, Medizinische Klinik II, Klinikstrasse 36, 35392 Giessen, Germany
Biochem J 380:385-92. 2004..This outcome suggests that disruption of both M and P loci would significantly increase susceptibility to airway infections, particularly those associated with colonization by Gram-positive organisms...
Expression of ABCA3 in developing lung and other tissuesMildred T Stahlman
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
J Histochem Cytochem 55:71-83. 2007..The role of ABCA3 in extrapulmonary tissues and organs remains to be elucidated. This manuscript contains online supplemental material at (www.jhc.org). Please visit this article online to view these materials...
Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosisMartina Korfei
University of Giessen Lung Center, Department of Internal Medicine II, Klinikstrasse 36, 35392 Giessen, Germany
Am J Respir Crit Care Med 178:838-46. 2008..The molecular pathomechanisms underlying idiopathic pulmonary fibrosis (IPF) are elusive, but chronic epithelial injury has recently been suggested as key event...
