All Outputs (3)

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.