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Condition: Cystic Fibrosis

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Total 62 results found since Jan 2013.

Tartrate resistant acid phosphatase 5 (TRAP5) mediates immune cell recruitment in a murine model of pulmonary bacterial infection
In this study, we found that Trap5-/- mice had impaired clearance of P. aeruginosa airway infection and reduced recruitment of immune cells (i.e. neutrophils and inflammatory macrophages). Trap5 knockdown using siRNA resulted in a decreased activation of the proinflammatory transcription factor NF-κB in reporter mice and a subsequent decrease of proinflammatory gene expression. Add-back experiments of enzymatically active TRAP5 to Trap5-/- mice restored immune cell recruitment and bacterial killing. In human CF lung tissue, TRAP5 of alveolar macrophages was detected in proximity to OPN to a higher degree than in normal lu...
Source: Frontiers in Immunology - December 8, 2022 Category: Allergy & Immunology Source Type: research

Spray drying siRNA-lipid nanoparticles for dry powder pulmonary delivery
This study verifies the successful spray drying procedure of LNP-siRNA systems maintaining their integrity and mediating strong gene silencing efficiency on mRNA and protein levels both in vitro and ex vivo. The successful spray drying procedure of LNP-siRNA formulations in 5% lactose solution creates a novel siRNA-based therapy option to target respiratory diseases such as lung cancer, asthma, COPD, cystic fibrosis and viral infections.PMID:36126785 | DOI:10.1016/j.jconrel.2022.09.021
Source: Cancer Control - September 20, 2022 Category: Cancer & Oncology Authors: Christoph M Zimmermann Domizia Baldassi Karen Chan Nathan B P Adams Alina Neumann Diana Leidy Porras-Gonzalez Xin Wei Nikolaus Kneidinger Mircea Gabriel Stoleriu Gerald Burgstaller Dominik Witzigmann Paola Luciani Olivia M Merkel Source Type: research

The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Plays an Important Role in Fetal Human Colon Cell Migration and Proliferation
CONCLUSION-: Our results indicate that CFTR plays an important role in FHC colon cell migration and EGFR signaling, which could lead to a better understanding as to why CF patients have increased risk for colon cancer.PMID:35556394 | DOI:10.1096/fasebj.2022.36.S1.R6139
Source: Cancer Control - May 13, 2022 Category: Cancer & Oncology Authors: Cara A Mogan Garett J Grant Kaylee LeBaron Theodore G Liou My N Helms Source Type: research

GSE165718 BACH1, the master regulator of oxidative stress, modulates CFTR gene expression RNA-seq
Contributors : Monali NandyMazumdar ; Alekh Paranjapye ; James Browne ; Shih-Hsing Leir ; Shiyi Yin ; Ann HarrisSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensThe cystic fibrosis transmembrane conductance regulator (CFTR) gene lies within a TAD in which multiple cis-regulatory elements (CREs) and transcription factors (TFs) regulate its cell-specific expression. The CREs are recruited to the gene promoter by a looping mechanism that depends upon both architectural proteins and specific TFs. An siRNA screen to identify TFs coordinating CFTR expression in airway epithelial cells sugges...
Source: GEO: Gene Expression Omnibus - November 17, 2021 Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research

GSE165717 BACH1, the master regulator of oxidative stress, modulates CFTR gene expression ChIP-seq
Contributors : Monali NandyMazumdar ; Alekh Paranjapye ; James Brown ; Shiyi Yin ; Shih-Hsing Leir ; Ann HarrisSeries Type : Genome binding/occupancy profiling by high throughput sequencingOrganism : Homo sapiensThe cystic fibrosis transmembrane conductance regulator (CFTR) gene lies within a TAD in which multiple cis-regulatory elements (CREs) and transcription factors (TFs) regulate its cell-specific expression. The CREs are recruited to the gene promoter by a looping mechanism that depends upon both architectural proteins and specific TFs. An siRNA screen to identify TFs coordinating CFTR expression in airway epithelial...
Source: GEO: Gene Expression Omnibus - November 17, 2021 Category: Genetics & Stem Cells Tags: Genome binding/occupancy profiling by high throughput sequencing Homo sapiens Source Type: research

GSE165716 BACH1, the master regulator of oxidative stress, modulates CFTR gene expression 4C-seq
Contributors : Ann Harris ; Monali NandyMazumdar ; Shiy Yin ; Alekh Paranjapye ; James Brown ; Shih-Hsing LeirSeries Type : OtherOrganism : Homo sapiensThe cystic fibrosis transmembrane conductance regulator (CFTR) gene lies within a TAD in which multiple cis-regulatory elements (CREs) and transcription factors (TFs) regulate its cell-specific expression. The CREs are recruited to the gene promoter by a looping mechanism that depends upon both architectural proteins and specific TFs. An siRNA screen to identify TFs coordinating CFTR expression in airway epithelial cells suggested an activating role for BTB Domain and CNC H...
Source: GEO: Gene Expression Omnibus - November 17, 2021 Category: Genetics & Stem Cells Tags: Other Homo sapiens Source Type: research

BACH1, the master regulator of oxidative stress, has a dual effect on CFTR expression
Biochem J. 2021 Oct 4:BCJ20210252. doi: 10.1042/BCJ20210252. Online ahead of print.ABSTRACTThe cystic fibrosis transmembrane conductance regulator (CFTR) gene lies within a TAD in which multiple cis-regulatory elements (CREs) and transcription factors (TFs) regulate its cell-specific expression. The CREs are recruited to the gene promoter by a looping mechanism that depends upon both architectural proteins and specific TFs. An siRNA screen to identify TFs coordinating CFTR expression in airway epithelial cells suggested an activating role for BTB Domain and CNC Homolog 1 (BACH1). BACH1 is a ubiquitous master regulator of t...
Source: The Biochemical Journal - October 4, 2021 Category: Biochemistry Authors: Monali NandyMazumdar Alekh Paranjapye James Browne Shiyi Yin Shih-Hsing Leir Ann Harris Source Type: research

GSE131084 Coordinate regulation of ELF5 and EHF at the chr11p13 modifier region
Contributors : Ann Harris ; Shiyi Yin ; Jenny L KerschnerSeries Type : Genome binding/occupancy profiling by high throughput sequencingOrganism : Homo sapiensE74-like factor 5 (ELF5) and ETS-homologous factor (EHF) are epithelial selective ETS family transcription factors (TFs) encoded by genes at chr11p13, a region associated with cystic fibrosis (CF) lung disease severity. EHF controls many key processes in lung epithelial function so its regulatory mechanisms are important. Using CRISPR/Cas9 technology, we removed three cis-regulatory elements (CREs) from the chr11p13 region in airway epithelial cells. Deletion of two e...
Source: GEO: Gene Expression Omnibus - May 11, 2021 Category: Genetics & Stem Cells Tags: Genome binding/occupancy profiling by high throughput sequencing Homo sapiens Source Type: research

siRNA delivery to macrophages using aspherical, nanostructured microparticles as delivery system for pulmonary administration.
Abstract The delivery of oligonucleotides such as siRNA to the lung is a major challenge, as this group of drugs has difficulties to overcome biological barriers due to its polyanionic character and the associated hydrophilic properties, resulting in inefficient delivery. Especially in diseases such as asthma, chronic obstructive pulmonary disease and cystic fibrosis, where increased proinflammation is present, a targeted RNA therapy is desirable due to the high potency of these oligonucleotides. To address these problems and to ensure efficient uptake of siRNA in macrophages, a microparticulate, cylindrical deliv...
Source: European Journal of Pharmaceutics and Biopharmaceutics - December 4, 2020 Category: Drugs & Pharmacology Authors: Fischer T, Tschernig T, Drews F, Brix K, Meier C, Simon M, Kautenburger R, Schneider M Tags: Eur J Pharm Biopharm Source Type: research

TMEM16A Mediated Mucus Production in Human Airway Epithelial Cells.
We examined this question using BCi-NS1 cells, a human airway basal cell line that maintains multipotent differentiation capacity, and the two human airway epithelial cell lines, Calu-3 and CFBE. The data demonstrate that exposure of airway epithelial cells to IL-8 and IL-13, two cytokines known to be enhanced in CF and asthma, respectively, leads to increase in mucus production. Expression of MUC5AC was fully dependent on expression of TMEM16A, as shown by siRNA-knockdown of TMEM16A. In addition, different inhibitors of TMEM16A attenuated IL-13 induced mucus production. Interestingly, in CFBE cells expressing F508delCFTR,...
Source: American Journal of Respiratory Cell and Molecular Biology - October 7, 2020 Category: Molecular Biology Authors: Cabrita I, Benedetto R, Wanitchakool P, Lerias J, Centeio R, Ousingsawat J, Schreiber R, Kunzelmann K Tags: Am J Respir Cell Mol Biol Source Type: research

Increased intracellular Cl- concentration improves airway epithelial migration by activating the RhoA/ROCK Pathway
Conclusion: Taken together, these results suggested that Cl- accumulation in airway epithelial cells could activate the RhoA/ROCK/LIMK cascade to induce F-actin reorganization, down-regulate cell stiffness, and improve epithelial migration.
Source: Theranostics - October 3, 2020 Category: Molecular Biology Authors: Wenjie Huang, Meiling Tan, Yue Wang, Lei Liu, Yan Pan, Jingjing Li, Mingxing Ouyang, Chunjiao Long, Xiangping Qu, Huijun Liu, Chi Liu, Jia Wang, Linhong Deng, Yang Xiang, Xiaoqun Qin Tags: Research Paper Source Type: research

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Human Lung Microvascular Endothelial Cells Controls Oxidative Stress, Reactive Oxygen-Mediated Cell Signaling and Inflammatory Responses
ConclusionFunctional endothelial CFTR limits oxidative stress and contributes to the normal anti-inflammatory state of HLMVECs. Therapeutic strategies to restore endothelial CFTR function in CF are warranted.
Source: Frontiers in Physiology - July 28, 2020 Category: Physiology Source Type: research

Genes, Vol. 11, Pages 351: Extracellular Vesicle-Mediated siRNA Delivery, Protein Delivery, and CFTR Complementation in Well-Differentiated Human Airway Epithelial Cells
L. Sinn Extracellular vesicles (EVs) are a class of naturally occurring secreted cellular bodies that are involved in long distance cell-to-cell communication. Proteins, lipids, mRNA, and miRNA can be packaged into these vesicles and released from the cell. This information is then delivered to target cells. Since EVs are naturally adapted molecular messengers, they have emerged as an innovative, inexpensive, and robust method to deliver therapeutic cargo in vitro and in vivo. Well-differentiated primary cultures of human airway epithelial cells (HAE) are refractory to standard transfection techniques. Indeed, common s...
Source: Genes - March 25, 2020 Category: Genetics & Stem Cells Authors: Brajesh K. Singh Ashley L. Cooney Sateesh Krishnamurthy Patrick L. Sinn Tags: Article Source Type: research