Professor of Respiratory Medicine, Faculty of Medicine & Health Sciences
- 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 Brendan Mallia-Millanes Clinical Research Fellow: Proteolytic mechanisms of lung destruction in COPD
Dr Shamsa Naveed Clinical Research Fellow Functional effects of MMP-1 in asthma
Jaineeta Richardson PhD student: Co-culture models of IPF
Chris Philp PhD student: ECM stability in IPF
Arundhati Dongre PhD student: Proteases in LAM
CLEMENTS, D., MARKWICK, L.J., PURI, N. and JOHNSON, S.R., 2010. Role of the CXCR4/CXCL12 axis in lymphangioleiomyomatosis and angiomyolipoma Journal of Immunology. 185(3), 1812–1821 JOHNSON, S R, CORDIER, J F, LAZOR, R, COTTIN, V, COSTABEL, U, HARARI, S, REYNAUD-GAUBERT, M, BOEHLER, A, BRAUNER, M, POPPER, H, BONETTI, F, KINGSWOOD, C and ,, 2010. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. The European respiratory journal. 35(1), 14-26
DAVIES, D.M., DE VRIES, P.J., JOHNSON, S.R., MCCARTNEY, D.L., COX, J.A., SERRA, A.L., WATSON, P.C., HOWE, C.J., DOYLE, T., POINTON, K., CROSS, J.J., TATTERSFIELD, A.E., KINGSWOOD, J.C. and SAMPSON, J.R., 2011. Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: a phase 2 trial Clinical Cancer Research: an official journal of the American Association for Cancer Research. 17(June), 4071-4081