Research Topics
Genomes and Genes | James P BridgesSummaryAffiliation: Cincinnati Children's Hospital Medical Center Country: USA Publications
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Detail Information
Publications
Conditional hypoxia inducible factor-1α induction in embryonic pulmonary epithelium impairs maturation and augments lymphangiogenesisJames P Bridges
Perinatal Institute, Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
Dev Biol 362:24-41. 2012..Collectively, these data indicate that HIF-1a protein levels in the pulmonary epithelium must be tightly controlled for proper development of the epithelial and mesenchymal compartments...
Meckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein CMei Wang
Division of Pulmonary Biology, Cincinnati Children s Research Foundation, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229 3039, USA
J Biol Chem 284:33377-83. 2009..These results support a model in which MKS3 links the ER lumenal quality control machinery with the cytosolic degradation apparatus...
ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein CMei Dong
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, and the University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA
Mol Biol Cell 19:2620-30. 2008..ERdj4 and ERdj5 promote turnover of misfolded SP-C and this activity is dependent on their ability to stimulate BiP ATPase activity...
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...
LPCAT1 regulates surfactant phospholipid synthesis and is required for transitioning to air breathing in miceJames 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...
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...
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...
