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Source: European Respiratory Journal
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Total 164 results found since Jan 2013.
Haploid genetic screening identifies a novel regulator of BMPR2
Pulmonary arterial hypertension (PAH), is characterised by profound remodelling of small pulmonary arteries, leading to increased pulmonary arterial pressures and premature death by right heart failure. Heterozygous germ-line mutations in the bone morphogenetic protein type II receptor (BMPR2) cause ~70% of familial PAH and ~20% of idiopathic PAH cases. The majority of mutations lead to haploinsufficiency but crucially, regardless of the presence of mutation, lung BMPR2 expression is reduced in all forms of PAH. Therefore, restoration of BMPR2 levels is an important therapeutic target. We previously showed that BMPR2 ubiqu...
Source: European Respiratory Journal - October 28, 2020 Category: Respiratory Medicine Authors: Dunmore, B., Burr, S., Upton, P., Nathan, J., Morrell, N. Tags: Pulmonary hypertension Source Type: research
Protein truncating mutations in ATP13A3 promote pulmonary arterial hypertension
Conclusions: Our initial findings provide further evidence that loss of function mutations in ATP13A3 are directly involved in causation of PAH and the mechanism involves alterations in cellular polyamine levels.
Source: European Respiratory Journal - October 28, 2020 Category: Respiratory Medicine Authors: Legchenko, E., Liu, B., West, J., Vangheluwe, P., Upton, P., Morrell, N. Tags: Pulmonary hypertension Source Type: research
Novel KLF4-FOXO1 Interactome in Lung Fibroblasts:Regulator of Myofibroblasts in Hyperoxia-Induced Lung Injury
Conclusion: Our data unveil a novel Klf4-FoxO1 axis in lung fibroblasts that could promote matrix remodeling and lung growth arrest in lungs with BPD.
Source: European Respiratory Journal - October 28, 2020 Category: Respiratory Medicine Authors: Hirani, D., Koch, M., Mohr, J., Vohlen, C., Nayakanti, S., Grimm, C., Schweiger, M.-R., Georgomanolis, T., Pullamsetti, S., Dötsch, J., Alejandre Alcazar, M. A. Tags: Lung and airway developmental biology Source Type: research
Late Breaking Abstract - Involvement of Orai1 Ca2+ channel in the pathogenesis of pulmonary arterial hypertension. Orai1 as a new potential therapeutic target ?
Conclusion: Altogether our results provide proof of concept that Orai1 should be considered as a new therapeutic target in PAH.
Source: European Respiratory Journal - November 25, 2021 Category: Respiratory Medicine Authors: Masson, B., Le Ribeuz, H., Sabourin, J., Woodhouse, E., Foster, R., Ruchon, Y., Dutheil, M., Boët, A., Ghigna, M.-R., Mercier, O., Beech, D., Benitah, J.-P., Bailey, M., Humbert, M., Montani, D., Capuano, V., Antigny, F. Tags: Pulmonary hypertension Source Type: research
Mitochondrial DNA stress activates MHC class I antigen presentation and CD8+ T-cell immunity: implications for pulmonary fibrosis
Mitochondrial dysfunction is a hallmark of IPF and promotes the development of fibrosis. Mitochondrial stress has been shown to activate innate immunity via mitochondrial (mt) DNA sensing. However, whether mitochondrial stress regulates adaptive immune responses is unknown. We here investigated the regulation of MHC I antigen presentation by mitochondrial stress using a chronic mitochondrial dysfunction and an acute mtDNA stress model.Mouse embryonic fibroblasts derived from the PolgA mutator mouse, which represents a system of disturbed mitochondrial function, demonstrated concerted upregulation of the MHC I antigen prese...
Source: European Respiratory Journal - November 25, 2021 Category: Respiratory Medicine Authors: Wang, X., Meul, T., Kammerl, I., Wang, Y., Mayr, C., Schiller, H. B., Meiners, S. Tags: Airway cell biology and immunopathology Source Type: research
LSC - 2021 - COL4A3 expression in asthmatic epithelium depends on intronic methylation and ZNF263 binding
Conclusion: Our data suggests an epigenetic modification at cg11797365 interrupts ZNF263 binding which may contribute to the COL4A3 expression deficit in asthmatic airways.
Source: European Respiratory Journal - November 25, 2021 Category: Respiratory Medicine Authors: Sai Sneha Priya, N., Joseph Vermeulen, C., Pech, M., Faiz, A., George G. Oliver, B., Van Den Berge, M., Kay Burgess, J., Kopp, M., Weckmann, M. Tags: Occupational and environmental health Source Type: research
UPF1 and STAU1 modulate rhinovirus pathophysiology contributing to impaired antiviral immunity in asthma
Conclusions: Decreased UPF1 levels in asthma BECs underlie their impaired antiviral immunity. We demonstrate for the first time the interplay between two novel factors, UPF1 and STAU1, in RV pathophysiology and induced exacerbations.
Source: European Respiratory Journal - November 25, 2021 Category: Respiratory Medicine Authors: Martinez-Nunez, R. T., Richardson, A., Ponde, N., Bagley, D., Ortiz-Zapater, E., Rynne, J., Howarth, P., Rosenblatt, J., Edwards, M. Tags: Airway cell biology and immunopathology Source Type: research
Screening and verification of differentially expressed genes for airway remodeling in asthma
Conclusion: The expression of CTSK may related to the airway remodeling of asthma by up-regulating the TGF-β1 expression in BECs.
Source: European Respiratory Journal - December 1, 2022 Category: Respiratory Medicine Authors: Qin, L., Liu, C., Wu, M., Yao, Y. Tags: 05.03 - Allergy and immunology Source Type: research
Extracellular vesicles (EVs) as diagnostic tools in the phenotypic determination of lung tumors
Immunotherapy has been one of the great advances in recent years for the treatment of advanced tumors. Our goal is to determine whether extracellular vesicles (EVs) are effective and appropriate tools for detecting and blocking immune checkpoints proteins (ICPs). We developed a method for tracing tumor content via an analysis of EVs surface makers in the bloodstream using the MAGPIX platform (Fig.1). As a proof of concept, we have developed the detection of 4 ICP-EV. We will use this technology to monitor the ICP-EV profile in patients undergoing immunotherapeutic treatment. Second, endothelial EVs will be used as drug car...
Source: European Respiratory Journal - December 1, 2022 Category: Respiratory Medicine Authors: Cambier, M., Muller, F., Henket, M., Remacle, C., Guiot, J., Struman, I. Tags: 11.01 - Lung cancer Source Type: research
Prolyl-3-hydroxylase 4 (P3H4), a novel player in collagen synthesis and secretion in lung fibroblasts
In conclusion, P3H4 and FKBP10 reciprocally regulate each other in lung fibroblasts, and P3H4 represents a novel modulator of collagen biosynthesis in the lung and hence a potential therapeutic target for IPF.
Source: European Respiratory Journal - December 1, 2022 Category: Respiratory Medicine Authors: Onursal, C., Knüppel, L., Lietman, C. D., Merl-Pham, J., Hatz, R., Behr, J., Unger, K., Hess, J., Hauck, S. M., Lee, B., Staab-Weijnitz, C. Tags: 12.01 - Idiopathic interstitial pneumonias Source Type: research
Inhibitory role of retinoic acid-related orphan receptor alpha RORa in human lung fibroblast activation.
We examined the role of RORα in human lung fibroblasts (HLF) from controls (n=4) and IPF (n=5). siRNA knockdown of RORα resulted in a significant upregulation of aSMA protein in IPF and control HLFs. Treatment with RORa antagonist R1001 revealed a trend of upregulation of aSMA mRNA, while, an opposite trend was observed with RORa agonist, R1078 in IPF and control HLFs. Furthermore, the induction of aSMA levels in IPF HLFs treated with R1001 was significantly higher compared to treated control HLFs. Next, the effect of TGFb1 treatment on RORa expression was evaluated. Importantly, a significant downregulati...
Source: European Respiratory Journal - December 1, 2022 Category: Respiratory Medicine Authors: Zevla, D. M., Tsitoura, E., Crestani, B., Antoniou, K. Tags: 12.01 - Idiopathic interstitial pneumonias Source Type: research
MiR-125b-5p up-regulation in asthma and COPD overlap patients mediates oxidative stress and apoptosis via targeting IL6R/NLRC5/TRIAP1
Conclusions: MiR-125b-5p is up-regulated in ACO patients, and may cause oxidative stress and cell apoptosis through targeting IL6R, NLRC5, TRIAP1 genes.
Source: European Respiratory Journal - December 1, 2022 Category: Respiratory Medicine Authors: Chang, Y., Lee, C., Chen, Y. Tags: 03.01 - Molecular pathology and functional genomics Source Type: research
COX2 expression is downregulated in a shear stress magnitude-dependent manner in BMPR2-silenced HPAECs: implications for prostacyclin deficiency in PAH
Conclusion: BMPR2 loss may disrupt the flow-driven production of COX2 in HPAECs, which may be an underlying mechanism of endothelial dysfunction in PAH.
Source: European Respiratory Journal - December 1, 2022 Category: Respiratory Medicine Authors: Mahomed, A., Burke-Gaffney, A., Ghazaly, M., Naser, J., Toe, Q., Quinlan, G., Wort, S. J. Tags: 13.01 - Pulmonary hypertension Source Type: research
Investigating the effects of metals within particulate matter in human alveolar epithelial cells
Conclusion: These novel data show the ability of metals present in PM to modulate a range of signalling pathways to elicit differential cellular responses and activate several potentially pathological mechanisms. Future work will further explore these mechanisms.
Source: European Respiratory Journal - December 1, 2022 Category: Respiratory Medicine Authors: Dean, L. S., Vethakan-Raj, H. S., Parkin, J. G., Ridley, R. A., Conforti, F., Downward, J., Wang, Y., Jones, M. G., Davies, D. E., Loxham, M. Tags: 03.02 - Airway cell biology and immunopathology Source Type: research
