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List of works by Simon R. Johnson

A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis.

scientific article published on 5 December 2013

A 4-year prospective evaluation of protocols to improve clinical outcomes for patients with lymphangioleiomyomatosis in a national clinical centre

scientific article

A theoretical model of inflammation- and mechanotransduction-driven asthmatic airway remodelling

scientific article published on 02 July 2018

Accelerated extracellular matrix turnover during exacerbations of COPD.

scientific article published on 11 June 2015

Air travel in women with lymphangioleiomyomatosis.

scientific article

An assay to evaluate the long term effects of inflammatory mediators on airway smooth muscle: evidence that TNF(alpha)up-regulates 5-HT(2A) mediated contraction

scientific article published on December 2002

Analysis of the oestrogen response in an angiomyolipoma derived xenograft model

scientific article

Association of MMP-2 polymorphisms with severe and very severe COPD: a case control study of MMPs-1, 9 and 12 in a European population

scientific article

CCR3 induced-p42/44 MAPK activation protects against staurosporine induced-DNA fragmentation but not apoptosis in airway smooth muscle cells.

scientific article published in July 2012

Cardiovascular and inflammatory effects of simvastatin therapy in patients with COPD: a randomized controlled trial

scientific article

Cathepsin K in Lymphangioleiomyomatosis: LAM Cell-Fibroblast Interactions Enhance Protease Activity by Extracellular Acidification.

scientific article

Chronic Respiratory Diseases. Rare lung diseases. Introduction

scientific article published in January 2005

Clinical experience of lymphangioleiomyomatosis in the UK. ⬇️

scientific article published on December 2000

Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis.

scientific article

Collagen I and thrombin activate MMP-2 by MMP-14-dependent and -independent pathways: implications for airway smooth muscle migration.

scientific article published on 22 December 2006

Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use

scientific article published on 30 July 2019

Discoidin domain receptor 1 regulates bronchial epithelial repair and matrix metalloproteinase production

scientific article published on 30 September 2010

ECM Crosslinking Enhances Fibroblast Growth and Protects Against Matrix Proteolysis in Lung Fibrosis.

scientific article

Effect of doxycycline on proliferation, MMP production, and adhesion in LAM-related cells

scientific article published on 25 June 2010

Emerging clinical picture of lymphangioleiomyomatosis.

scientific article published on 29 July 2005

Evolution of lung pathology in lymphangioleiomyomatosis: associations with disease course and treatment response

scientific article published on 30 April 2020

Extra-cellular matrix proteins induce matrix metalloproteinase-1 (MMP-1) activity and increase airway smooth muscle contraction in asthma

scientific article published on 28 February 2014

Fungal recognition enhances mannose receptor shedding through dectin-1 engagement

scientific article

In search of the fibrotic epithelial cell: opportunities for a collaborative network.

scientific article published on 15 June 2011

Interstitial lung disease following certolizumab pegol.

scientific article published on 23 November 2011

Levels of circulating MMP-7 degraded elastin are elevated in pulmonary disorders.

scientific article published on 9 July 2015

Living with lymphangioleiomyomatosis

scientific article

Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study

scientific article

Lymphangioleiomyomatosis

scientific article published on 01 May 2006

Lymphangioleiomyomatosis

scientific article published on 01 September 2016

Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline.

scientific article

MMP-9 protein level does not reflect overall MMP activity in the airways of patients with COPD.

scientific article published on 6 March 2008

Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis

scientific article published on 10 December 2020

Matrix Metalloproteinase-1 Activation Contributes to Airway Smooth Muscle Growth and Asthma Severity.

scientific article published on 14 December 2016

Matrix metalloproteinase expression and activity in human airway smooth muscle cells.

scientific article published on 20 July 2004

Matrix metalloproteinase-12 (MMP-12) SNP affects MMP activity, lung macrophage infiltration and protects against emphysema in COPD.

scientific article published on 5 July 2011

Matrix metalloproteinases -8 and -9 in the Airways, Blood and Urine During Exacerbations of COPD.

scientific article

Natural history of angiomyolipoma in lymphangioleiomyomatosis: implications for screening and surveillance

scientific article

Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management

scientific article published in September 2016

Perspectives for improving the evaluation and access of therapies for rare lung diseases in Europe

scientific article published on 11 March 2012

Pneumothorax and the biology of Birt-Hogg-Dubé syndrome

scientific article published on 01 June 2020

Pregnancy experiences among women with lymphangioleiomyomatosis

scientific article published on 30 December 2008

Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts

scientific article published on 11 August 2020

Presence of a prothrombotic state in people with idiopathic pulmonary fibrosis: a population-based case-control study.

scientific article published on 3 September 2013

Reply to: Treatment of Pulmonary Lymphangioleiomyomatosis During Pregnancy.

scientific article published on 22 January 2018

Residual Lung Abnormalities after COVID-19 Hospitalization: Interim Analysis of the UKILD Post–COVID-19 Study

scientific article published in 2023

Role of the CXCR4/CXCL12 axis in lymphangioleiomyomatosis and angiomyolipoma

scientific article published on 28 June 2010

Secretory leukocyte protease inhibitor gene deletion alters bleomycin-induced lung injury, but not development of pulmonary fibrosis

scientific article published on 14 March 2016

Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: a phase 2 trial

scientific article published on 27 April 2011

Sirolimus therapy in tuberous sclerosis or sporadic lymphangioleiomyomatosis

scientific article

Sphingolipid, fatty acid and phospholipid metabolites are associated with disease severity and mTOR inhibition in lymphangioleiomyomatosis

scientific article published on 28 May 2020

Study of breast cancer incidence in patients of lymphangioleiomyomatosis.

scientific article published on 7 March 2016

Subcellular distribution of the TSC2 gene product tuberin in human airway smooth muscle cells is driven by multiple localization sequences and is cell-cycle dependent

scientific article published on 11 August 2006

Survival and disease progression in UK patients with lymphangioleiomyomatosis.

scientific article

Synthetic functions of airway smooth muscle in asthma

scientific article

TAILS proteomics reveals dynamic changes in airway proteolysis controlling protease activity and innate immunity during COPD exacerbations

scientific article published on 04 October 2018

TIMP-1 in asthma: guilty by association

scientific article published in August 2005

The ERS guidelines for LAM: trying a rationale approach to a rare disease

scientific article published on 06 May 2010

The International LAM Registry: a component of an innovative web-based clinician, researcher, and patient-driven rare disease research platform.

scientific article published on March 2010

The TSC-2 product tuberin is expressed in lymphangioleiomyomatosis and angiomyolipoma.

scientific article

The UK guidelines for management and surveillance of Tuberous Sclerosis Complex

scientific article published on 01 March 2019

The clinical profile of tuberous sclerosis complex (TSC) in the United Kingdom: A retrospective cohort study in the Clinical Practice Research Datalink (CPRD).

scientific article published on 11 December 2015

The economic burden of tuberous sclerosis complex in the UK: A retrospective cohort study in the Clinical Practice Research Datalink

scientific article

The role of extracellular matrix quality in pulmonary fibrosis.

scientific article published on 29 October 2014

The role of inflammation resolution speed in airway smooth muscle mass accumulation in asthma: insight from a theoretical model

scientific article

Tissue and matrix influences on airway smooth muscle function

scientific article published on 24 December 2008

Untangling the protease web in COPD: metalloproteinases in the silent zone

scientific article published on 01 February 2016

Use of a three-dimensional cell culture model to study airway smooth muscle-mast cell interactions in airway remodeling

scientific article published on 3 April 2009

Wild type mesenchymal cells contribute to the lung pathology of lymphangioleiomyomatosis

scientific article published on 15 May 2015

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