scholarly journals Endoplasmic Reticulum Stress Induced by Surfactant Protein C BRICHOS Mutants Promotes Proinflammatory Signaling by Epithelial Cells

2011 ◽  
Vol 44 (3) ◽  
pp. 404-414 ◽  
Author(s):  
Jean Ann Maguire ◽  
Surafel Mulugeta ◽  
Michael F. Beers
Keyword(s):  
Endoplasmic Reticulum ◽  
Epithelial Cells ◽  
Endoplasmic Reticulum Stress ◽  
Protein C ◽  
Surfactant Protein ◽  
Surfactant Protein C

scholarly journals A Surfactant Protein C Precursor Protein BRICHOS Domain Mutation Causes Endoplasmic Reticulum Stress, Proteasome Dysfunction, and Caspase 3 Activation

2005 ◽  
Vol 32 (6) ◽  
pp. 521-530 ◽  
Author(s):  
Surafel Mulugeta ◽  
Vu Nguyen ◽  
Scott J. Russo ◽  
Madesh Muniswamy ◽  
Michael F. Beers
Keyword(s):  
Endoplasmic Reticulum ◽  
Endoplasmic Reticulum Stress ◽  
Protein C ◽  
Caspase 3 ◽  
Surfactant Protein ◽  
Precursor Protein ◽  
Surfactant Protein C

scholarly journals Meckel-Gruber Syndrome Protein MKS3 Is Required for Endoplasmic Reticulum-associated Degradation of Surfactant Protein C

2009 ◽  
Vol 284 (48) ◽  
pp. 33377-33383 ◽  
Author(s):  
Mei Wang ◽  
James P. Bridges ◽  
Cheng-Lun Na ◽  
Yan Xu ◽  
Timothy E. Weaver
Keyword(s):  
Endoplasmic Reticulum ◽  
Protein C ◽  
Surfactant Protein ◽  
Surfactant Protein C ◽  
Endoplasmic Reticulum Associated Degradation ◽  
Syndrome Protein

scholarly journals ERdj4 and ERdj5 Are Required for Endoplasmic Reticulum-associated Protein Degradation of Misfolded Surfactant Protein C

2008 ◽  
Vol 19 (6) ◽  
pp. 2620-2630 ◽  
Author(s):  
Mei Dong ◽  
James P. Bridges ◽  
Karen Apsley ◽  
Yan Xu ◽  
Timothy E. Weaver
Keyword(s):  
Endoplasmic Reticulum ◽  
Protein Degradation ◽  
Transient Expression ◽  
Protein C ◽  
Binding Protein ◽  
Surfactant Protein ◽  
Wild Type ◽  
Surfactant Protein C ◽  
Er Retention ◽  
Specific Association

Mutations in the SFTPC gene associated with interstitial lung disease in human patients result in misfolding, endoplasmic reticulum (ER) retention, and degradation of the encoded surfactant protein C (SP-C) proprotein. In this study, genes specifically induced in response to transient expression of two disease-associated mutations were identified by microarray analyses. Immunoglobulin heavy chain binding protein (BiP) and two heat shock protein 40 family members, endoplasmic reticulum-localized DnaJ homologues ERdj4 and ERdj5, were significantly elevated and exhibited prolonged and specific association with the misfolded proprotein; in contrast, ERdj3 interacted with BiP, but it did not associate with either wild-type or mutant SP-C. Misfolded SP-C, ERdj4, and ERdj5 coprecipitated with p97/VCP indicating that the cochaperones remain associated with the misfolded proprotein until it is dislocated to the cytosol. Knockdown of ERdj4 and ERdj5 expression increased ER retention and inhibited degradation of misfolded SP-C, but it had little effect on the wild-type protein. Transient expression of ERdj4 and ERdj5 in X-box binding protein 1−/− mouse embryonic fibroblasts substantially restored rapid degradation of mutant SP-C proprotein, whereas transfection of HPD mutants failed to rescue SP-C endoplasmic reticulum-associated protein degradation. ERdj4 and ERdj5 promote turnover of misfolded SP-C and this activity is dependent on their ability to stimulate BiP ATPase activity.

scholarly journals Selective targeting of alveolar type II respiratory epithelial cells by anti-surfactant protein-C antibody-conjugated lipoplexes

2015 ◽  
Vol 203 ◽  
pp. 140-149 ◽  
Author(s):  
Yun Wu ◽  
Junyu Ma ◽  
Parker S. Woods ◽  
Nicholas M. Chesarino ◽  
Chang Liu ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Protein C ◽  
Surfactant Protein ◽  
Alveolar Type ◽  
Type Ii ◽  
Respiratory Epithelial Cells ◽  
Surfactant Protein C ◽  
Selective Targeting ◽  
Respiratory Epithelial

scholarly journals Regulation of p53-mediated changes in the uPA-fibrinolytic system and in lung injury by loss of surfactant protein C expression in alveolar epithelial cells

2017 ◽  
Vol 312 (6) ◽  
pp. L783-L796 ◽  
Author(s):  
Bijesh Puthusseri ◽  
Amarnath Marudamuthu ◽  
Nivedita Tiwari ◽  
Jian Fu ◽  
Steven Idell ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Protein C ◽  
Surfactant Protein ◽  
Alveolar Epithelial Cells ◽  
Caveolin 1 ◽  
Surfactant Protein C ◽  
Alveolar Epithelial ◽  
Upa System ◽  
Deficient Mice ◽  
Pai 1

Pulmonary surfactant protein C (SP-C) expression by type II alveolar epithelial cells (AECs) is markedly reduced in diverse types of lung injuries and is often associated with AEC apoptosis. It is unclear whether loss of SP-C contributes to the increased p53 and urokinase-type plasminogen activator (uPA) system cross-talk and apoptosis of AECs. Therefore, we inhibited SP-C expression in human and murine AECs using lentivirus vector expressing shRNA and tested p53 and downstream changes in the uPA-fibrinolytic system. Inhibition of SP-C expression in AECs induced p53 and activated caspase-3, indicating AEC apoptosis. We also found that bleomycin or cigarette smoke exposure failed to inhibit SP-C expression or apoptosis in AECs in p53- and plasminogen activator inhibitor-1 (PAI-1)-deficient mice. Depletion of SP-C expression by lentiviral SP-C shRNA in PAI-1-deficient mice failed to induce p53 or apoptosis in AECs, whereas it increased both AEC p53 and apoptosis in wild-type and uPA-deficient mice. SP-C inhibition in AECs also increased in CXCL1 and CXCL2 and their receptor CXCR2 as well as ICAM-1 expression, which is indicative of a proinflammatory response. Overexpression of p53-binding 3′-UTR sequences in AECs inhibited PAI-1 induction while maintaining uPA and uPAR protein and mRNA expression. Furthermore, caveolin-1 expression and phosphorylation were increased in AECs, indicating an intricate link between caveolin-1 and Src kinase-mediated cell signaling and AEC apoptosis due to loss of SP-C expression through p53 and uPA system-mediated cross-talk. The role of uPA, PAI-1, and p53 in the regulation of AEC apoptosis after injury was also determined in knockout mice.

HDAC inhibitors may regulate EMT and surfactant protein C in human alveolar epithelial cells

2015 ◽  
Author(s):  
Vijay D'Souza ◽  
Domokos Bartis ◽  
Aaron Scott ◽  
Fang Gao Smith ◽  
David Thickett
Keyword(s):  
Epithelial Cells ◽  
Protein C ◽  
Hdac Inhibitors ◽  
Surfactant Protein ◽  
Alveolar Epithelial Cells ◽  
Surfactant Protein C ◽  
Alveolar Epithelial ◽  
Human Alveolar Epithelial Cells

scholarly journals Synthetic Processing of Surfactant Protein C by Alevolar Epithelial Cells

1998 ◽  
Vol 273 (24) ◽  
pp. 15287-15293 ◽  
Author(s):  
Michael F. Beers ◽  
Catherine A. Lomax ◽  
Scott J. Russo
Keyword(s):  
Epithelial Cells ◽  
Protein C ◽  
Surfactant Protein ◽  
Surfactant Protein C
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