All Outputs (25)

Elevated PLAUR is observed in the airway epithelium of asthma patients and blocking improves barrier integrity (2023)
Journal Article
Portelli, M. A., Bhaker, S., Pang, V., Bates, D. O., Johnson, S. R., Mazar, A. P., …Sayers, I. (2023). Elevated PLAUR is observed in the airway epithelium of asthma patients and blocking improves barrier integrity. Clinical and Translational Allergy, 13(10), Article e12293. https://doi.org/10.1002/clt2.12293

AbstractBackgroundExpression of the urokinase plasminogen activator receptor (uPAR) is elevated in the airway epithelium in asthma; however, the contribution of uPAR to asthma pathogenesis and scope for therapeutic targeting remains unknown.Objective... Read More about Elevated PLAUR is observed in the airway epithelium of asthma patients and blocking improves barrier integrity.

Lysyl oxidase like 2 is increased in asthma and contributes to asthmatic airway remodelling (2022)
Journal Article
Ramis, J., Middlewick, R., Pappalardo, F., Cairns, J. T., Stewart, I. D., John, A. E., …Tatler, A. L. (2022). Lysyl oxidase like 2 is increased in asthma and contributes to asthmatic airway remodelling. European Respiratory Journal, 60(1), Article 2004361. https://doi.org/10.1183/13993003.04361-2020

BACKGROUND: Airway smooth muscle (ASM) cells are fundamental to asthma pathogenesis, influencing bronchoconstriction, airway hyperresponsiveness and airway remodelling. The extracellular matrix (ECM) can influence tissue remodelling pathways; however... Read More about Lysyl oxidase like 2 is increased in asthma and contributes to asthmatic airway remodelling.

Human bronchial epithelial cells from patients with asthma have an altered gene expression profile (2022)
Journal Article
Bhaker, S., Portelli, M. A., Rakkar, K., Shaw, D., Johnson, S., Brightling, C., & Sayers, I. (2022). Human bronchial epithelial cells from patients with asthma have an altered gene expression profile. ERJ Open Research, 8(1), Article 0625-2021. https://doi.org/10.1183/23120541.00625-2021Published21February2022

Cross talk between LAM cells and fibroblasts may influence alveolar epithelial cell behavior in lymphangioleiomyomatosis (2022)
Journal Article
Clements, D., Miller, S., Babaei-Jadidi, R., Adam, M., Potter, S. S., & Johnson, S. R. (2022). Cross talk between LAM cells and fibroblasts may influence alveolar epithelial cell behavior in lymphangioleiomyomatosis. AJP - Lung Cellular and Molecular Physiology, 322(2), L283-L293. https://doi.org/10.1152/AJPLUNG.00351.2021

Lymphangioleiomyomatosis (LAM) is a female-specific cystic lung disease in which tuberous sclerosis complex 2 (TSC2)-deficient LAM cells, LAM-associated fibroblasts (LAFs), and other cell types infiltrate the lungs. LAM lesions can be associated with... Read More about Cross talk between LAM cells and fibroblasts may influence alveolar epithelial cell behavior in lymphangioleiomyomatosis.

Understanding the burden of interstitial lung disease post-COVID-19: the UK Interstitial Lung Disease-Long COVID Study (UKILD-Long COVID) (2021)
Journal Article
Wild, J. M., Porter, J. C., Molyneaux, P. L., George, P. M., Stewart, I., Allen, R. J., …Jenkins, R. G. (2021). Understanding the burden of interstitial lung disease post-COVID-19: the UK Interstitial Lung Disease-Long COVID Study (UKILD-Long COVID). BMJ Open Respiratory Research, 8(1), Article e001049. https://doi.org/10.1136/bmjresp-2021-001049

Introduction The COVID-19 pandemic has led to over 100 million cases worldwide. The UK has had over 4 million cases, 400 000 hospital admissions and 100 000 deaths. Many patients with COVID-19 suffer long-term symptoms, predominantly breathlessness a... Read More about Understanding the burden of interstitial lung disease post-COVID-19: the UK Interstitial Lung Disease-Long COVID Study (UKILD-Long COVID).

Mast Cell Tryptase Release Contributes to Disease Progression in Lymphangioleiomyomatosis (2021)
Journal Article
Babaei-Jadidi, R., Dongre, A., Miller, S., Castellanos Uribe, M., Stewart, I. D., Thompson, Z. M., …Johnson, S. R. (2021). Mast Cell Tryptase Release Contributes to Disease Progression in Lymphangioleiomyomatosis. American Journal of Respiratory and Critical Care Medicine, 204(4), 431-444. https://doi.org/10.1164/rccm.202007-2854OC

Rationale: Lymphangioleiomyomatosis (LAM) is a multisystem disease that causes lung cysts and respiratory failure. Loss of TSC (tuberous sclerosis complex) gene function results in a clone of “LAM cells” with dysregulated mTOR (mechanistic target of... Read More about Mast Cell Tryptase Release Contributes to Disease Progression in Lymphangioleiomyomatosis.

Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis (2020)
Journal Article
Chernbumroong, S., Johnson, J., Gupta, N., Miller, S., Mccormack, F. X., Garibaldi, J. M., & Johnson, S. R. (2021). Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis. European Respiratory Journal, 57(6), Article 2003036. https://doi.org/10.1183/13993003.03036-2020

Background: Lymphangioleiomyomatosis (LAM) is a rare multisystem disease with variable clinical manifestations and differing rates of progression that make management decisions and giving prognostic advice difficult. We used machine learning to ident... Read More about Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis.

Phenotypic and functional translation of IL33 genetics in asthma (2020)
Journal Article
Ketelaar, M. E., Portelli, M. A., Dijk, F. N., Shrine, N., Faiz, A., Vermeulen, C. J., …Nawijn, M. C. (2021). Phenotypic and functional translation of IL33 genetics in asthma. Journal of Allergy and Clinical Immunology, 147(1), 144-157. https://doi.org/10.1016/j.jaci.2020.04.051

© 2020 Background: Asthma is a complex disease with multiple phenotypes that may differ in disease pathobiology and treatment response. IL33 single nucleotide polymorphisms (SNPs) have been reproducibly associated with asthma. IL33 levels are elevate... Read More about Phenotypic and functional translation of IL33 genetics in asthma.

Phenotypic and functional translation of IL1RL1 locus polymorphisms in lung tissue and asthmatic airway epithelium (2020)
Journal Article
Portelli, M. A., Dijk, F. N., Ketelaar, M. E., Shrine, N., Hankinson, J., Bhaker, S., …Sayers, I. (2020). Phenotypic and functional translation of IL1RL1 locus polymorphisms in lung tissue and asthmatic airway epithelium. JCI insight, 5(8), Article 132446. https://doi.org/10.1172/jci.insight.132446

The IL1RL1 (ST2) gene locus is robustly associated with asthma; however, the contribution of single nucleotide polymorphisms (SNPs) in this locus to specific asthma subtypes and the functional mechanisms underlying these associations remain to be def... Read More about Phenotypic and functional translation of IL1RL1 locus polymorphisms in lung tissue and asthmatic airway epithelium.

Airway epithelial cell isolation techniques affect DNA methylation profiles with consequences for analysis of asthma related perturbations to DNA methylation (2019)
Journal Article
Clifford, R. L., Patel, J., MacIsaac, J. L., McEwen, L. M., Johnson, S. R., Shaw, D., …Kobor, M. S. (2019). Airway epithelial cell isolation techniques affect DNA methylation profiles with consequences for analysis of asthma related perturbations to DNA methylation. Scientific Reports, 9(1), 1-12. https://doi.org/10.1038/s41598-019-50873-y

The airway epithelium forms the interface between the inhaled environment and the lung. The airway epithelium is dysfunctional in asthma and epigenetic mechanisms are considered a contributory factor. We hypothesised that the DNA methylation profiles... Read More about Airway epithelial cell isolation techniques affect DNA methylation profiles with consequences for analysis of asthma related perturbations to DNA methylation.

Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use (2019)
Journal Article
Johnson, J., & Johnson, S. R. (2019). Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use. Thorax, 74(10), 999-1002. https://doi.org/10.1136/thoraxjnl-2019-213338

Lymphangioleiomyomatosis can be associated with reversible airflow obstruction and although no guidelines around reversibility testing or inhaled therapy exist, many patients receive bronchodilators and inhaled corticosteroids. To better identify tho... Read More about Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use.

Accelerated 19F·MRI Detection of Matrix Metalloproteinase-2/-9 through Responsive Deactivation of Paramagnetic Relaxation Enhancement (2019)
Journal Article
Faas, H. M., Krupa, J. L., Taylor, A. J., Zamberlan, F., Philp, C. J., Williams, H. E., …Meersmann, T. (2019). Accelerated 19F·MRI Detection of Matrix Metalloproteinase-2/-9 through Responsive Deactivation of Paramagnetic Relaxation Enhancement. Contrast Media and Molecular Imaging, 2019, 1-13. https://doi.org/10.1155/2019/4826520

Paramagnetic gadolinium ions (Gd III), complexed within DOTA-based chelates, have become useful tools to increase the magnetic resonance imaging (MRI) contrast in tissues of interest. Recently, " on/off " probes serving as 19 F·MRI biosensor for targ... Read More about Accelerated 19F·MRI Detection of Matrix Metalloproteinase-2/-9 through Responsive Deactivation of Paramagnetic Relaxation Enhancement.

TAILS proteomics reveals dynamic changes in airway proteolysis controlling protease activity and innate immunity during COPD exacerbations (2018)
Journal Article
Mallia-Milanes, B., Dufour, A., Philp, C., Solis, N., Klein, T., Fischer, M., …Johnson, S. R. (2018). TAILS proteomics reveals dynamic changes in airway proteolysis controlling protease activity and innate immunity during COPD exacerbations. AJP - Lung Cellular and Molecular Physiology, 315(6), L1003-L1014. https://doi.org/10.1152/ajplung.00175.2018

© 2018 the American Physiological Society. Dys-regulated protease activity is thought to cause parenchymal and airway damage in chronic obstructive pulmonary disease (COPD). Multiple proteases have been implicated in COPD, and identifying their subst... Read More about TAILS proteomics reveals dynamic changes in airway proteolysis controlling protease activity and innate immunity during COPD exacerbations.

The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis (2018)
Journal Article
Miller, S., Coveney, C., Johnson, J., Farmaki, A. E., Gupta, N., Tobin, M. D., …Johnson, S. R. (2018). The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis. European Respiratory Journal, 52(5), Article 1801074. https://doi.org/10.1183/13993003.00951-2018

Background: Lymphangioleiomyomatosis (LAM) is a rare disease of women. Decline in lung function is variable making appropriate targeting of therapy difficult. We used unbiased serum proteomics to identify markers associated with outcome in LAM.
Meth... Read More about The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis.

Endocrine therapy for breast cancer: a model of hormonal manipulation (2018)
Journal Article
Johnston, S. J., & Cheung, K.-L. (in press). Endocrine therapy for breast cancer: a model of hormonal manipulation. Oncology and Therapy, https://doi.org/10.1007/s40487-018-0062-x

Oestrogen receptor (ER) is the driving transcription factor in 70% of breast cancer. Endocrine therapies targeting the ER represent one of the most successful anticancer strategies to date. In the clinic, novel targeted agents are now being exploited... Read More about Endocrine therapy for breast cancer: a model of hormonal manipulation.

A theoretical model of inflammation- and mechanotransduction-driven asthmatic airway remodelling (2018)
Journal Article
Hill, M., Philp, C. J., Billington, C. K., Tatler, A. L., Johnson, S. R., O'Dea, R. D., & Brook, B. S. (2018). A theoretical model of inflammation- and mechanotransduction-driven asthmatic airway remodelling. Biomechanics and Modeling in Mechanobiology, 17(5), 1451-1470. https://doi.org/10.1007/s10237-018-1037-4

© 2018, The Author(s). Inflammation, airway hyper-responsiveness and airway remodelling are well-established hallmarks of asthma, but their inter-relationships remain elusive. In order to obtain a better understanding of their inter-dependence, we de... Read More about A theoretical model of inflammation- and mechanotransduction-driven asthmatic airway remodelling.

ECM crosslinking enhances fibroblast growth and protects against matrix proteolysis in lung fibrosis (2017)
Journal Article
Philp, C. J., Siebeke, I., Clements, D., Miller, S., Habgood, A., John, A. E., …Johnson, S. R. (2018). ECM crosslinking enhances fibroblast growth and protects against matrix proteolysis in lung fibrosis. American Journal of Respiratory Cell and Molecular Biology, 58(5), 594–603. https://doi.org/10.1165/rcmb.2016-0379OC

Idiopathic pulmonary fibrosis (IPF) is characterised by accumulation of extra cellular matrix (ECM) proteins and fibroblast proliferation. ECM cross-linking enzymes have been implicated in fibrotic diseases and we hypothesised that the ECM in IPF is... Read More about ECM crosslinking enhances fibroblast growth and protects against matrix proteolysis in lung fibrosis.

Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study (2017)
Journal Article
Bee, J., Fuller, S., Miller, S., & Johnson, S. R. (2018). Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study. Thorax, 73(4), 369-375. https://doi.org/10.1136/thoraxjnl-2017-210872

Rationale

Mechanistic target of rapamycin inhibitors reduce loss of lung function in lymphangioleiomyomatosis (LAM), although their benefit varies between individuals. We examined lung function response and side effects to rapamycin in a national... Read More about Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study.

Cathepsin K in lymphangioleiomyomatosis: LAM cell-fibroblast Interactions enhance protease activity by extracellular acidification (2017)
Journal Article
Dongre, A., Clements, D., Fisher, A. J., & Johnson, S. R. (2017). Cathepsin K in lymphangioleiomyomatosis: LAM cell-fibroblast Interactions enhance protease activity by extracellular acidification. American Journal of Pathology, 187(8), 1750-1762. https://doi.org/10.1016/j.ajpath.2017.04.014

Lymphangioleiomyomatosis (LAM) is a rare disease in which clonal ‘LAM’ cells infiltrate the lungs and lymphatics. In association with recruited fibroblasts, LAM cells form nodules adjacent to lung cysts. It is assumed LAM nodule derived proteases lea... Read More about Cathepsin K in lymphangioleiomyomatosis: LAM cell-fibroblast Interactions enhance protease activity by extracellular acidification.

MMP-1 activation contributes to airway smooth muscle growth and asthma severity (2016)
Journal Article
Naveed, S.-U.-N., Clements, D., Jackson, D. J., Philp, C., Billington, C. K., Soomro, I. N., Reynolds, C., Harrison, T. W., Johnston, S. L., Shaw, D. E., & Johnson, S. R. (2017). MMP-1 activation contributes to airway smooth muscle growth and asthma severity. American Journal of Respiratory and Critical Care Medicine, 195(8), 1000-1009. https://doi.org/10.1164/rccm.201604-0822OC

Introduction: Matrix metalloproteinase-1 and mast cells are present in the airways of people with asthma. We hypothesised that matrix metalloproteinase-1 could be activated by mast cells and increase asthma severity. Methods: Patients with stable ast... Read More about MMP-1 activation contributes to airway smooth muscle growth and asthma severity.