[Cite as http://purl.org/au-research/grants/nhmrc/303137]
A/Pr Steven Mutsaers
Dr Mary-Anne Kedda
Prof Darryl Knight
Brief description Pulmonary fibrosis is a chronic diffuse interstitial lung disease of unknown cause, characterised pathologically by inflammation and fibrosis of the lung tissue. The prognosis is poor with a 50% mortality at five years after diagnosis and considerable morbidity during those years. Previous investigations have documented the role for inflammation in the development of pulmonary fibrosis and current therapeutic strategies are aimed at suppressing the inflammation. Data generated over the past decade also have established the concept that the molecular processes underlying the fibrogenesis component may represent a new opportunity for therapeutic intervention. Attempts to treat fibrosis have for the most part consisted of anti- inflammatory drugs, almost exclusively steroids. The effectiveness of steroids is variable and can be associated with significant side effects. This project will examine the effects of a family of molecules called cytokines that signal through gp130 as critical determinants of disease susceptibility and progression. gp 130 is a shared component in the receptor complexes for IL-6 family cytokines (IL-6, IL-11, LIF, OSM) which are important regulators of both the phenotype and proliferation of fibroblasts in health and in response to injury. Our data raises the possibility of developing pharmacological manipulators of gp130 signalling pathways that would suppress fibrosis but leave normal cellular defense mechanisms necessary for host defense in the lung intact.
Funding Amount $AUD 456,500.00
Funding Scheme NHMRC Project Grants
Standard Project Grant