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Condition: Cystic Fibrosis

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Total 62 results found since Jan 2013.

A functional tandem between transient receptor potential canonical channels 6 and calcium-dependent chloride channels in Human epithelial cells.
In conclusion, we show that TRPC6 channel is pivotal for the activation of CaCC by guanabenz through a α2-adrenergic-independent pathway in human airway epithelial cells. We suggest propose a functional coupling between TRPC6 and CaCC and guanabenz as a potential TRPC6 activator for exploring TRPC6 and CaCC channel functions and corresponding channelopathies. PMID: 26265544 [PubMed - as supplied by publisher]
Source: European Journal of Pharmacology - August 8, 2015 Category: Drugs & Pharmacology Authors: Bertrand J, Dannhoffer L, Antigny F, Vachel L, Jayle C, Vandebrouck C, Becq F, Norez C Tags: Eur J Pharmacol Source Type: research

Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection Research Communication
We sought to determine the mechanisms by which influenza infection of human epithelial cells decreases cystic fibrosis transmembrane conductance regulator (CFTR) expression and function. We infected human bronchial epithelial (NHBE) cells and murine nasal epithelial (MNE) cells with various strains of influenza A virus. Influenza infection significantly reduced CFTR short circuit currents (Isc) and protein levels at 8 hours postinfection. We then infected CFTR expressing human embryonic kidney (HEK)-293 cells (HEK-293 CFTRwt) with influenza virus encoding a green fluorescent protein (GFP) tag and performed whole-cell and c...
Source: FASEB Journal - June 30, 2015 Category: Biology Authors: Londino, J. D., Lazrak, A., Noah, J. W., Aggarwal, S., Bali, V., Woodworth, B. A., Bebok, Z., Matalon, S. Tags: Research Communication Source Type: research

Role of cystic fibrosis transmembrane conductance regulator-associated ligand (CAL) in regulating the trafficking and signaling of corticotropin-releasing factor receptor 1.
Abstract Corticotropin releasing factor (CRF) receptor1 (CRFR1) is associated with psychiatric illness and is a proposed target for the treatment of anxiety and depression. Like many G protein-coupled receptors (GPCRs), CRFR1 harbors a PDZ (PSD-95/Disc Large/Zona Occludens 1)-binding motif at the end of its carboxyl terminal tail. The interactions of PDZ proteins with GPCRs is crucial for the regulation of their receptor function. In the present study, we characterize the interaction of the cystic fibrosis transmembrane conductance regulator-associated ligand (CAL) with CRFR1. We show using co-immunoprecipitation ...
Source: Cellular Signalling - June 23, 2015 Category: Cytology Authors: Hammad MM, Dunn HA, Walther C, Ferguson SS Tags: Cell Signal Source Type: research

Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity
by Firhan A. Malik, Anja Meissner, Illya Semenkov, Steven Molinski, Stan Pasyk, Saumel Ahmadi, Hai H. Bui, Christine E. Bear, Darcy Lidington, Steffen-Sebastian Bolz The cystic fibrosis transmembrane conductance regulator (CFTR) attenuates sphingosine-1-phosphate (S1P) signaling in resistance arteries and has emerged as a prominent regulator of myogenic vasoconstriction. This investigation demonstrates that S1P inhibits CFTR activity via adenosine monophosphate-activated kinase (AMPK), establishing a potential feedback link. In Baby Hamster Kidney (BHK) cells expressing wild-type human CFTR, S1P (1μmol/L) attenuates fors...
Source: PLoS One - June 16, 2015 Category: Biomedical Science Authors: Firhan A. Malik et al. Source Type: research

Pro-resolving Action of MAG-DHA in Lung Inflammatory Models Related to Cystic Fibrosis.
CONCLUSIONS: MAG-DHA displays anti-inflammatory properties and reduces mucin overexpression in Calu-3 cells and human bronchi untreated or treated with P. aeruginosa LPS, a finding consistent with the effects of ResolvinD1, a known anti-inflammatory mediator. PMID: 25781052 [PubMed - as supplied by publisher]
Source: Am J Respir Cell Mol... - March 17, 2015 Category: Respiratory Medicine Authors: Morin C, Cantin AM, Rousseau É, Sirois M, Sirois C, Rizcallah E, Fortin S Tags: Am J Respir Cell Mol Biol Source Type: research

Induction of heme oxygenase-1 contributes to survival of Mycobacterium abscessus in human macrophages-like THP-1 cells
Publication date: Available online 20 January 2015 Source:Redox Biology Author(s): Maher Y. Abdalla , Iman M. Ahmad , Barbara Switzer , Bradley E. Britigan Mycobacterium abscessus (M.abs) is a rapidly growing mycobacterial species that infects macrophages, and is an important pathogen in patients with cystic fibrosis. We studied the early stages of M.abs infection of macrophages, with emphasis on the role of heme-oxygenase-1 (HO-1) in this infection. THP-1 cells were activated using TPA into macrophage-like cells and infected with M.abs for different time points. M.abs infection robustly induced HO-1 expression in the TH...
Source: Redox Biology - January 23, 2015 Category: Biology Source Type: research

The low PLC-δ1 expression in cystic fibrosis bronchial epithelial cells induces upregulation of TRPV6 channel activity.
This study revealed TRPV6 and PLC-δ1 as critical actor of Ca(2+) homeostasis in CF human bronchial epithelial cells. PMID: 25477137 [PubMed - as supplied by publisher]
Source: Cell Calcium - November 18, 2014 Category: Cytology Authors: Vachel L, Norez C, Jayle C, Becq F, Vandebrouck C Tags: Cell Calcium Source Type: research

Impaired TLR4 and HIF expression in cystic fibrosis bronchial epithelial cells downregulates hemeoxygenase-1 and alters iron homeostasis in vitro
In this study, we investigated in vitro the role of Toll-like receptor 4 (TLR4), hypoxia-inducible factor 1α (HIF-1α), and iron on HO-1 expression in cystic fibrosis (CF). Immunohistochemical analysis of TLR4, HO-1, ferritin, and HIF-1α were performed on lung sections of CFTR–/– and wild-type mice. CFBE41o- and 16HBE14o- cell lines were employed for in vitro analysis via immunoblotting, immunofluorescence, real-time PCR, luciferase reporter gene analysis, and iron quantification. We observed a reduced TLR4, HIF-1α, HO-1, and ferritin in CFBE41o- cell line and CF mice. Knockdown studies u...
Source: AJP: Lung Cellular and Molecular Physiology - November 15, 2014 Category: Respiratory Medicine Authors: Chillappagari, S., Venkatesan, S., Garapati, V., Mahavadi, P., Munder, A., Seubert, A., Sarode, G., Guenther, A., Schmeck, B. T., Tummler, B., Henke, M. O. Tags: ARTICLES Source Type: research

ERp29 regulates epithelial sodium channel functional expression by promoting channel cleavage
The epithelial Na+ channel (ENaC) plays a key role in the regulation of blood pressure and airway surface liquid volume. ERp29 is a 29-kDa thioredoxin-homologous endoplasmic reticulum (ER) protein that has only a single cysteine instead of the usual thioredoxin CXXC motif. Our group previously demonstrated that ERp29 promotes biogenesis of the cystic fibrosis transmembrane conductance regulator (CFTR). On the basis of similarities of CFTR and ENaC trafficking, we hypothesized that ERp29 would also regulate ENaC biogenesis and functional expression. In epithelial cells, overexpression of wild-type (wt) ERp29 increased ENaC ...
Source: AJP: Cell Physiology - October 15, 2014 Category: Cytology Authors: Grumbach, Y., Bikard, Y., Suaud, L., Chanoux, R. A., Rubenstein, R. C. Tags: ARTICLES Source Type: research

Impaired TLR4 and HIF expression in cystic fibrosis bronchial epithelial cells downregulates hemeoxygenase-1 and alters iron homeostasis in vitro.
In this study, we investigated in vitro the role of toll-like receptor 4 (TLR4), hypoxia-inducible factor-1α (HIF-1α) and iron on HO-1 expression in cystic fibrosis (CF). Immunohistochemical analysis of TLR4, HO-1, ferritin and HIF-1α were performed on lung sections of CFTR-/- and wildtype mice. CFBE41o- and 16HBE14o- cell lines were employed for in vitro analysis via immunoblotting, immunofluorescence, real-time PCR, luciferase reporter gene analysis and iron quantification. We observed a reduced TLR4, HIF-1α, HO-1, and ferritin in CFBE41o- cell line and CF mice. Knockdown studies using TLR4-siRNA in 16HBE14o- reveale...
Source: American Journal of Physiology. Lung Cellular and Molecular Physiology - September 19, 2014 Category: Cytology Authors: Chillappagari S, Venkatesan S, Garapati V, Mahavadi P, Munder A, Seubert A, Sarode G, Guenther A, Schmeck BT, Tümmler B, Henke MO Tags: Am J Physiol Lung Cell Mol Physiol Source Type: research

Architectural proteins CTCF and cohesin have distinct roles in modulating the higher order structure and expression of the CFTR locus
Higher order chromatin structures across the genome are maintained in part by the architectural proteins CCCTC binding factor (CTCF) and the cohesin complex, which co-localize at many sites across the genome. Here, we examine the role of these proteins in mediating chromatin structure at the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CFTR encompasses nearly 200 kb flanked by CTCF-binding enhancer-blocking insulator elements and is regulated by cell-type-specific intronic enhancers, which loop to the promoter in the active locus. SiRNA-mediated depletion of CTCF or the cohesin component, RAD21, showed ...
Source: Nucleic Acids Research - September 1, 2014 Category: Research Authors: Gosalia, N., Neems, D., Kerschner, J. L., Kosak, S. T., Harris, A. Tags: Gene regulation, Chromatin and Epigenetics Source Type: research

ERp29 Regulates Epithelial Sodium Channel Functional Expression by Promoting Channel Cleavage.
Abstract The Epithelial Na(+) Channel (ENaC) plays a key role in the regulation of blood pressure and airway surface liquid volume. ERp29 is a thioredoxin-homologous endoplasmic reticulum protein of 29 kD that has only a single Cysteine instead of the usual thioredoxin C-X-X-C motif. Our group has previously demonstrated that ERp29 promotes biogenesis of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Based on similarities of CFTR and ENaC trafficking, we hypothesized that ERp29 would also regulate ENaC biogenesis and functional expression. In epithelial cells, overexpression of wt ERp29 increased ...
Source: Am J Physiol Cell Ph... - June 18, 2014 Category: Cytology Authors: Grumbach Y, Bikard Y, Suaud L, Chanoux RA, Rubenstein RC Tags: Am J Physiol Cell Physiol Source Type: research

SGK1 and Shank2E Cell Biology
The glucocorticoid dexamethasone increases cystic fibrosis transmembrane conductance regulator (CFTR) abundance in human airway epithelial cells by a mechanism that requires serum- and glucocorticoid-induced protein kinase 1 (SGK1) activity. The goal of this study was to determine whether SGK1 increases CFTR abundance by phosphorylating Shank2E, a PDZ domain protein that contains two SGK1 phosphorylation consensus sites. We found that SGK1 phosphorylates Shank2E as well as a peptide containing the first SGK1 consensus motif of Shank2E. The dexamethasone-induced increase in CFTR abundance was diminished by overexpression of...
Source: Journal of Biological Chemistry - June 12, 2014 Category: Chemistry Authors: Koeppen, K., Coutermarsh, B. A., Madden, D. R., Stanton, B. A. Tags: Molecular Bases of Disease Source Type: research

Cx26 regulates proliferation of repairing basal airway epithelial cells.
We report that induction of Cx26 in repairing HAECs is associated with cell proliferation. We also show that Cx26 is expressed in a population of CK14-positive basal-like cells. Cx26 silencing in immortalized cell lines using siRNA and in primary HAECs using lentiviral-transduced shRNA enhanced Ki67-labeling index and Ki67 mRNA, indicating that Cx26 acts a negative regulator of HAEC proliferation. Cx26 silencing also markedly decreased the transcription of KLF4 in immortalized HAECs. We further show that CF HAECs exhibited deregulated expression of KLF4, Ki67 and Cx26 as well enhanced rate of wound closure in the early res...
Source: The International Journal of Biochemistry and Cell Biology - February 22, 2014 Category: Biochemistry Authors: Crespin S, Bacchetta M, Saab JB, Tantilipikorn P, Bellec J, Dudez T, Nguyen T, Kwak BR, Lacroix JS, Huang S, Wiszniewski L, Chanson M Tags: Int J Biochem Cell Biol Source Type: research

High-Content siRNA Screen Reveals Global ENaC Regulators and Potential Cystic Fibrosis Therapy Targets
Joana Almaça, Diana Faria, Marisa Sousa, Inna Uliyakina, Christian Conrad, Lalida Sirianant, Luka A. Clarke, José Paulo Martins, Miguel Santos, Jean-Karim Heriché, Wolfgang Huber, Rainer Schreiber, Rainer Pepperkok, Karl Kunzelmann, Margarida D. Amaral. Dysfunction of ENaC, the epithelial sodium channel that regulates salt and water reabsorption in epithelia, causes several human diseases, including cystic fibrosis (CF). To develop a global under....
Source: Cell - September 12, 2013 Category: Cytology Source Type: research