- Director National Centre for Lymphangioleiomyomatosis
- Co-chair of the European Respiratory Society LAM Task Force
- Member of the LAM Foundation scientific board
- Member of the Tuberous Sclerosis Association specialist advisory panel
Respiratory medicine; Interstitial lung disease; Lymphangioleiomyomatosis; Rare lung disease.
Airway remodelling in asthma is categorised by airway smooth muscle hypertrophy and hyperplasia, change in the type and amount of the extra cellular matrix and epithelial shedding and mucous gland hyperplasia. Our interest is how airway proteases and myofibroblast / extra cellular matrix interactions effect myofibroblast growth and behaviour. These studies employ primary airway cell culture, co-cultures, protein expression, functional activity assay real time PCR and proteomics.
Lymphangioleiomyomatosis (LAM) is a rare lung and lymphatic disease categorised by infiltration of smooth muscle type cells in the lungs and lymphatics leading to progressive respiratory impairment. The disease only effects women and is caused by a defect in one of the two proteins associated with tuberous sclerosis, tuberin and hamartin. Our group has a clinical interest in LAM, runs the UK LAM database and is involved in a number of clinical studies and trials.
Laboratory research projects on LAM include the mechanisms of lung destruction in LAM, prognostic biomarkers and the generation of model systems to study LAM.
Matrix metalloproteinases are proteolytic enzymes secreted by a range of cells and regulated by pro-inflammatory cytokines, growth factors, and extra cellular matrix. We have been studying these in airway myofibroblasts and also specific diseases including chronic obstructive pulmonary disease and LAM.
Metalloproteinases in lung remodelling, Molecular pathology and translational studies in lymphangioleiomyomatosis, COPD, asthma and interstitial lung disease.
Dr Debbie Clements Research Fellow: Pathogenesis of LAM, molecular biology
Dr Suzanne Miller Research Fellow: Biomarkers in LAM
Dr Charlotte Billington Research Fellow: Airway remodelling in asthma
Dr Chris Philp Research Fellow: Airway Remodelling, Proteases in COPD, ECM stability in IPF
Dr Roya Babaei-Jadidi Research Fellow: Inflammatory cells and modelling in LAM
Dr Marlies Fisher Research Fellow: Proteolysis in COPD
NAVEED SU, CLEMENTS D, JACKSON DJ, PHILP C, BILLINGTON CK, SOOMRO I, REYNOLDS C, HARRISON TW, JOHNSTON SL, SHAW DE and JOHNSON SR, 2016. Matrix Metalloproteinase-1 Activation Contributes to Airway Smooth Muscle Growth and Asthma Severity. American journal of respiratory and critical care medicine. 195(8), 1000-1009 PHILP, CJ, SIEBEKE, I, CLEMENTS, D, HABGOOD, A, JOHN, A, NAVARATNAM, V, HUBBARD, RB, JENKINS, G and JOHNSON, SR, 2017. Extra-cellular matrix cross-linking enhances fibroblast growth and protects against matrix proteolysis in lung fibrosis. American Journal of Respiratory Cell and Molecular Biology.
DAVIES DM, JOHNSON SR, TATTERSFIELD A, KINGSWOOD JC, COX JA, MCCARTNEY DL, DOYLE T, ELMSLIE F, SAGGAR A, DE VRIES P, SAMPSON JR., 2008. Sirolimus therapy for renal angiomyolipoma in patients with tuberous sclerosis or sporadic lymphangioleiomyomatosis. New England Journal of Medicine. 358, 200-3