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Condition: Cystic Fibrosis

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Total 62 results found since Jan 2013.

A transcription factor network represses CFTR gene expression in airway epithelial cells.
Abstract Mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR ) gene cause the inherited disorder cystic fibrosis (CF). Lung disease is the major cause of CF morbidity, though CFTR expression levels are substantially lower in the airway epithelium than in pancreatic duct and intestinal epithelia, which also show compromised function in CF. Recently developed small molecule therapeutics for CF are highly successful for one specific CFTR mutation and may have a positive impact on others. However, the low abundance of CFTR transcripts in the airway limits the opportunity for drugs to correct th...
Source: The Biochemical Journal - March 23, 2018 Category: Biochemistry Authors: Mutolo MJ, Leir SH, Fossum SL, Browne JA, Harris A Tags: Biochem J Source Type: research

Cellular mechanism for herbal medicine Junchoto to facilitate intestinal Cl − /water secretion that involves cAMP-dependent activation of CFTR
AbstractConstipation is a common symptom frequently compromising the quality of daily life. Several mechanistically different drugs have been used to mitigate constipation, including Japanese herbal (Kampo) medicines. However, the mechanisms of their actions are often not well understood. Here we aimed to investigate the molecular mechanisms underlying the effects of Junchoto (JCT), a Kampo medicine empirically prescribed for chronic constipation. Cl− channel activity was measured by the patch-clamp method in human cystic fibrosis transmembrane conductance regulator (CFTR)-expressing HEK293T cells and human intestinal Ca...
Source: Journal of Natural Medicines - March 22, 2018 Category: Drugs & Pharmacology Source Type: research

High-throughput screening identifies FAU protein as a regulator of mutant cystic fibrosis transmembrane conductance regulator channel Cell Biology
In cystic fibrosis, deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel causes misfolding and premature degradation. One possible approach to reducing the detrimental health effects of cystic fibrosis could be the identification of proteins whose suppression rescues F508del-CFTR function in bronchial epithelial cells. However, searches for these potential targets have not yet been conducted, particularly in a relevant airway background using a functional readout. To identify proteins associated with F508del-CFTR processing, we used a high-throughput functi...
Source: Journal of Biological Chemistry - January 26, 2018 Category: Chemistry Authors: Valeria Tomati, Emanuela Pesce, Emanuela Caci, Elvira Sondo, Paolo Scudieri, Monica Marini, Felice Amato, Giuseppe Castaldo, Roberto Ravazzolo, Luis J. V. Galietta, Nicoletta Pedemonte Tags: Molecular Bases of Disease Source Type: research

Retinoic acid promotes stem cell differentiation and embryonic development by transcriptionally activating CFTR
Publication date: Available online 9 January 2018 Source:Biochimica et Biophysica Acta (BBA) - Molecular Cell Research Author(s): Xiaofeng Li, Kin Lam Fok, Jinghui Guo, Yan Wang, Zhenqing Liu, Ziyi Chen, Chengdong Wang, Ye Chun Ruan, Sidney Siubun Yu, Hui Zhao, Ji Wu, Xiaohua Jiang, Hsiao Chang Chan Retinoic acid (RA) plays a pivotal role in many cellular processes; however, the signaling mechanisms mediating the effect of RA are not fully understood. Here, we show that RA transcriptionally upregulates cystic fibrosis transmembrane conductance regulator (Cftr) by promoting the direct binding of its receptor RARα to Cftr ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Cell Research - January 9, 2018 Category: Molecular Biology Source Type: research

Retinoic acid promotes stem cell differentiation and embryonic development by transcriptionally activating CFTR.
Abstract Retinoic acid (RA) plays a pivotal role in many cellular processes; however, the signaling mechanisms mediating the effect of RA are not fully understood. Here, we show that RA transcriptionally upregulates cystic fibrosis transmembrane conductance regulator (Cftr) by promoting the direct binding of its receptor RARα to Cftr promoter in mouse spermatogonia and embryonic stem (ES) cells. The RA/CFTR pathway is involved in the differentiation of spermatogonia and organogenesis during the embryo development of Xenopus laevis. Loss of CFTR by siRNA-mediated knockdown blunts the RA-induced spermatogonial diff...
Source: Biochimica et Biophysica Acta - January 8, 2018 Category: Biochemistry Authors: Li X, Fok KL, Guo J, Wang Y, Liu Z, Chen Z, Wang C, Ruan YC, Yu SS, Zhao H, Wu J, Jiang X, Chan HC Tags: Biochim Biophys Acta Source Type: research

Modulation of TMEM16A channel activity by the von Willebrand factor type A (VWA) domain of the calcium-activated chloride channel regulator 1 (CLCA1) Cell Biology
Calcium-activated chloride channels (CaCCs) are key players in transepithelial ion transport and fluid secretion, smooth muscle constriction, neuronal excitability, and cell proliferation. The CaCC regulator 1 (CLCA1) modulates the activity of the CaCC TMEM16A/Anoctamin 1 (ANO1) by directly engaging the channel at the cell surface, but the exact mechanism is unknown. Here we demonstrate that the von Willebrand factor type A (VWA) domain within the cleaved CLCA1 N-terminal fragment is necessary and sufficient for this interaction. TMEM16A protein levels on the cell surface were increased in HEK293T cells transfected with CL...
Source: Journal of Biological Chemistry - June 2, 2017 Category: Chemistry Authors: Monica Sala-Rabanal, Zeynep Yurtsever, Kayla N. Berry, Colin G. Nichols, Tom J. Brett Tags: Molecular Biophysics Source Type: research

O-Aminobenzoyl-S-Nitrosoglutathione: a Fluorogenic, Cell Permeable, Pseudo-Substrate for S-Nitrosoglutathione Reductase.
Abstract S-nitrosoglutathione reductase (GSNOR) is a multifunctional enzyme. It can catalyze NADH-dependent reduction of S-nitrosoglutathione (GSNO); as well as NAD(+)-dependent oxidation of hydroxymethylglutathione (HMGSH; an adduct formed by the spontaneous reaction between formaldehyde and glutathione). While initially recognized as the enzyme that is involved in formaldehyde detoxification, increasing amount of research evidence has shown that GSNOR also plays a significant role in nitric oxide mediated signaling through its modulation of protein S-nitrosothiol abundance via transnitrosation reactions with GSN...
Source: Free Radical Biology and Medicine - April 15, 2017 Category: Biology Authors: Sun BL, Palmer L, Alam SR, Adekoya I, Brown-Steinke K, Periasamy A, Mutus B Tags: Free Radic Biol Med Source Type: research

SYVN1, NEDD8, and FBXO2 Proteins Regulate {Delta}F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitin-mediated Proteasomal Degradation Cell Biology
This study provides new knowledge of the CFTR biosynthetic pathway. It suggests that SYVN1 and FBXO2 represent two distinct multiprotein complexes that may degrade ΔF508-CFTR in airway epithelia and identifies a new role for NEDD8 in regulating ΔF508-CFTR ubiquitination.
Source: Journal of Biological Chemistry - December 1, 2016 Category: Chemistry Authors: Shyam Ramachandran, Samantha R. Osterhaus, Kalpaj R. Parekh, Ashley M. Jacobi, Mark A. Behlke, Paul B. McCray, Jr. Tags: Molecular Bases of Disease Source Type: research

From Genesis To Revelation: The Role Of Inflammatory Mediators In Chronic Respiratory Diseases And Their Control By Nucleic Acid-Based Drugs.
Abstract Asthma, chronic obstructive pulmonary disease, cystic fibrosis, and idiopathic pulmonary fibrosis, are among the most common chronic diseases and their prevalence is increasing. Each of these diseases is characterized by the secretion of cytokines and pro-inflammatory molecules which are thought to play a critical role in their pathogenesis. Moreover, immune cells, particularly neutrophils, macrophages and dendritic cells as well structural cells such as epithelial and airway smooth muscle cells are also involved in the pathogenic cycle of these diseases. There is a pressing need for the development of ne...
Source: Current Drug Delivery - August 23, 2016 Category: Drugs & Pharmacology Authors: Di Gioia S, Sardo C, Castellani S, Porsio B, Belgiovine G, Carbone A, Giammona G, Cavallaro G Tags: Curr Drug Deliv Source Type: research

TRPA1 Channels Modulate Inflammatory Response in Respiratory Cells from Cystic Fibrosis Patients.
In conclusion, TRPA1 channels are involved in regulating the extent of airway inflammation driven by CF bronchial epithelial cells. PMID: 27281024 [PubMed - as supplied by publisher]
Source: Am J Respir Cell Mol... - June 8, 2016 Category: Respiratory Medicine Authors: Prandini P, De Logu F, Fusi C, Provezza L, Nassini R, Montagner G, Materazzi S, Munari S, Gilioli E, Bezzerri V, Finotti A, Lampronti I, Tamanini A, Dechecchi M, Lippi G, Ribeiro CM, Rimessi A, Pinton P, Gambari R, Geppetti P, Cabrini G Tags: Am J Respir Cell Mol Biol Source Type: research

Differential contribution of cis-regulatory elements to higher order chromatin structure and expression of the CFTR locus
Higher order chromatin structure establishes domains that organize the genome and coordinate gene expression. However, the molecular mechanisms controlling transcription of individual loci within a topological domain (TAD) are not fully understood. The cystic fibrosis transmembrane conductance regulator (CFTR) gene provides a paradigm for investigating these mechanisms. CFTR occupies a TAD bordered by CTCF/cohesin binding sites within which are cell-type-selective cis-regulatory elements for the locus. We showed previously that intronic and extragenic enhancers, when occupied by specific transcription factors, are recruite...
Source: Nucleic Acids Research - April 19, 2016 Category: Research Authors: Yang, R., Kerschner, J. L., Gosalia, N., Neems, D., Gorsic, L. K., Safi, A., Crawford, G. E., Kosak, S. T., Leir, S.-H., Harris, A. Tags: Gene regulation, Chromatin and Epigenetics Source Type: research

Chitosan as a non-viral co-transfection system in a cystic fibrosis cell line
This study shows proof-of-principle that co-transfection with chitosan might be an effective delivery system in a human CF cell line. It also offers a potential alternative to further develop therapeutic strategies for inherited disease treatments, such as CF. Graphical abstract
Source: International Journal of Pharmaceutics - February 22, 2016 Category: Drugs & Pharmacology Source Type: research

Augmentation of CFTR maturation by S-nitrosoglutathione reductase
S-nitrosoglutathione (GSNO) reductase regulates novel endogenous S-nitrosothiol signaling pathways, and mice deficient in GSNO reductase are protected from airways hyperreactivity. S-nitrosothiols are present in the airway, and patients with cystic fibrosis (CF) tend to have low S-nitrosothiol levels that may be attributed to upregulation of GSNO reductase activity. The present study demonstrates that 1) GSNO reductase activity is increased in the cystic fibrosis bronchial epithelial (CFBE41o–) cells expressing mutant F508del-cystic fibrosis transmembrane regulator (CFTR) compared with the wild-type CFBE41o– ce...
Source: AJP: Lung Cellular and Molecular Physiology - February 1, 2016 Category: Respiratory Medicine Authors: Zaman, K., Sawczak, V., Zaidi, A., Butler, M., Bennett, D., Getsy, P., Zeinomar, M., Greenberg, Z., Forbes, M., Rehman, S., Jyothikumar, V., DeRonde, K., Sattar, A., Smith, L., Corey, D., Straub, A., Sun, F., Palmer, L., Periasamy, A., Randell, S., Kelley Tags: CALL FOR PAPERS Source Type: research

Augmentation of CFTR maturation by S-nitrosoglutathione reductase.
Abstract S-Nitrosoglutathione reductase (GSNOR) regulates novel endogenous S-nitrosothiol signaling pathways, and mice deficient in GSNO reductase are protected from airways hyper-reactivity. S-Nitrosothiols are present in the airway, and in patients with cystic fibrosis (CF) tend to have low SNO levels that may be attributed to up-regulation of GSNO reductase activity. The present study demonstrates that (1) GSNO reductase activity is increased in the cystic fibrosis bronchial epithelial (CFBE41o(-) ) cells expressing mutant F508del-CFTR when compared to the wild type CFBE41o(-) cells, (2) GSNO reductase expressi...
Source: Am J Physiol Lung Ce... - December 4, 2015 Category: Respiratory Medicine Authors: Zaman K, Sawczak V, Zaidi A, Butler M, Bennett D, Getsy P, Zeinomar M, Greenberg Z, Forbes MS, Rehman S, Jyothikumar V, DeRonde K, Sattar A, Smith L, Corey DA, Straub A, Sun F, Palmer L, Periasamy A, Randell SH, Kelley TJ, Lewis SJ, Gaston B Tags: Am J Physiol Lung Cell Mol Physiol Source Type: research

Correctors Rescue CFTR Mutation {Delta}F508 Cell Biology
Correcting the processing of ΔF508-CFTR, the most common mutation in cystic fibrosis, is the major goal in the development of new therapies for this disease. Here, we determined whether ΔF508 could be rescued by a combination of small-molecule correctors, and identified the mechanism by which correctors rescue the trafficking mutant of cystic fibrosis transmembrane conductance regulator (CFTR). We transfected COS-7 cells with ΔF508, created HEK-293 stably expressing ΔF508, and utilized CFBE41o− cell lines stably transduced with ΔF508. As shown previously, ΔF508 expressed less protein, was unstable at physiological ...
Source: Journal of Biological Chemistry - October 16, 2015 Category: Chemistry Authors: Lopes-Pacheco, M., Boinot, C., Sabirzhanova, I., Morales, M. M., Guggino, W. B., Cebotaru, L. Tags: Molecular Bases of Disease Source Type: research