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Research Topic : airway fibrosis
Scheme : NHMRC Project Grants
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  • Funded Activity

    Understanding And Controlling Remodelling In Pulmonary Fibrosis And Asthma

    Funder
    National Health and Medical Research Council
    Funding Amount
    $431,839.00
    Summary
    The development of scar tissue is a normal response to tissue injury. When airway and lung tissue is injured by exposure to irritants, scarring greatly diminishes the function of the lung to allow transfer of oxygen to the tissue. In severe disease, the scarring may be fatal. We discovered that two factors involved in formation of scar tissue neutralise each other's effects. We are examining this interaction in human lung to develop new treatments for scarring-related lung diseases.
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    Funded Activity

    Fibulin-a Target For Lung Fibrosis?

    Funder
    National Health and Medical Research Council
    Funding Amount
    $571,429.00
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    Funded Activity

    Novel Candidate Genes, Lung Function And Allergic Airways Disease

    Funder
    National Health and Medical Research Council
    Funding Amount
    $581,892.00
    Summary
    We propose to study airway remodelling (structural changes to the airway) and in asthma using human samples and rodent models of asthma. We are particularly interested in investigating the role of trefoil peptide 2 and relaxin, two genes identified as determining lung function. To do this we need to understand the mechanisms of airway remodelling and its impact on disease severity in the patient. A strength of this study is availability of samples from a large study of human asthma.
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    Funded Activity

    Does The Pattern Of Ventilation Distribution Predict Airway Hyperresponsiveness?

    Funder
    National Health and Medical Research Council
    Funding Amount
    $256,973.00
    Summary
    The tendency for airways to narrow too easily when stimulated is called airway hyperresponsiveness (AHR). AHR is an important feature of asthma, but it also occurs in other diseases, such as chronic obstructive pulmonary disease (COPD) - an airway disease caused by smoking, and cystic fibrosis. People who have AHR have more severe respiratory disease, regardless of which disease they have, and are more likely to develop poor lung function in old age and to be hospitalised or die from their disea .... The tendency for airways to narrow too easily when stimulated is called airway hyperresponsiveness (AHR). AHR is an important feature of asthma, but it also occurs in other diseases, such as chronic obstructive pulmonary disease (COPD) - an airway disease caused by smoking, and cystic fibrosis. People who have AHR have more severe respiratory disease, regardless of which disease they have, and are more likely to develop poor lung function in old age and to be hospitalised or die from their disease. If we can understand the causes of AHR we will have a better understanding of why some people with respiratory disease have poor outcomes. We have recently discovered that, in asthma, there is a very close relationship between AHR and the uneven distribution of air within the lungs. We believe that structural changes in the airways that cause this uneven distribution make the airways prone to AHR. This raises the possibility that factors that cause uneven ventilation in other respiratory diseases might also predispose the airways to AHR. If this is true, it suggests that there is a single physiological basis for AHR in a range of different diseases, and would allow us to focus research more closely on the causes of uneven ventilation. In this project we will measure the relationship between AHR and uneven ventilation in people with asthma, COPD or cystic fibrosis. The study is important because older people with asthma, particularly those with permanently narrowed airways, are likely to have more structural changes in their airways than young asthmatics, whereas people with COPD and cystic fibrosis have a different pattern of both structural changes and airway inflammation from that in asthma. The study will tell us whether there is a consistent relationship between AHR and uneven ventilation. If so, this would be extremely strong evidence that the factors that cause uneven ventilation contribute to AHR, and will point the way to studies of new treatments.
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    Funded Activity

    MKP-1 As A Novel Anti-inflammatory Strategy In Asthma And Airway Remodelling

    Funder
    National Health and Medical Research Council
    Funding Amount
    $461,528.00
    Summary
    Asthma is a chronic disorder where airways are remodelled, or thickened, resulting in poor lung function. Airway remodelling is a consequence of long-term inflammation after multiple episodes of asthma. As the current drugs to treat remodelling have side effects, the aim of this grant is to investigate a novel anti-inflammatory strategy to reverse the development of airway remodelling by increasing the anti-inflammatory protein - MKP-1.
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    Funded Activity

    Regulation Of Inflammatory And Epithelial Responses In An Experimental Model Of Chronic Human Asthma

    Funder
    National Health and Medical Research Council
    Funding Amount
    $164,061.00
    Summary
    This project examines how chronic inflammation and scarring develop in the walls of the airways in asthma. The particular role of allergic mechanisms and of specific types of cells that are involved in allergic inflammation will be tested, using a much-improved mouse model of asthma. In this experimental model, which was developed by the investigators, sensitised mice are chronically exposed to low concentrations of aerosolised egg white protein. The proposed studies will involve comparisons wit .... This project examines how chronic inflammation and scarring develop in the walls of the airways in asthma. The particular role of allergic mechanisms and of specific types of cells that are involved in allergic inflammation will be tested, using a much-improved mouse model of asthma. In this experimental model, which was developed by the investigators, sensitised mice are chronically exposed to low concentrations of aerosolised egg white protein. The proposed studies will involve comparisons with animals that are genetically deficient in their ability to produce certain inflammation-related molecules, as well as with mice treated with antibodies to block the action of other such molecules.
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    Funded Activity

    Th17 Cell Cytokines In Airway Wall Remodelling In Chronic Asthma.

    Funder
    National Health and Medical Research Council
    Funding Amount
    $295,983.00
    Summary
    In asthma, structural changes in the airway wall occur which thicken the muscle and epithelial layers, stiffen the airways and increase mucus production. This 'remodelling' makes breathing more difficult and is not effectively reversed with current treatments. We will study the cells and molecules involved in the development of these changes. This project will increase our understanding of the processes which drive these changes and may lead to the development of improved medications.
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    Funded Activity

    Signal Transduction In Airway Remodelling

    Funder
    National Health and Medical Research Council
    Funding Amount
    $626,141.00
    Summary
    In the asthmatic lung structural changes, such as increased deposition of proteins which form the scaffolding of the airways (the extracellular matrix proteins), and an increased mass of bronchial smooth muscle cells occur. Many of these critical structural changes are not reversed or prevented with current asthma therapy, thus we need to investigate, by using lung cells and tissues , why they happen and how we can prevent them.
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    Funded Activity

    Epithelium-fibroblast Interactions In Response To Allergic Airway Inflammation

    Funder
    National Health and Medical Research Council
    Funding Amount
    $235,526.00
    Summary
    The airways of an asthmatic patient undergo dramatic structural changes over time. This remodelling is thought to be responsible for producing the changes in lung function that are frequently observed in someone with the disease. However, in contrast to normal wound repair, it is unclear why in the majority of asthmatics, inflammation leads to ongoing remodelling rather than a self limiting healing process. In this context, cells that line the airways (epithelium) as well as cells that sit immed .... The airways of an asthmatic patient undergo dramatic structural changes over time. This remodelling is thought to be responsible for producing the changes in lung function that are frequently observed in someone with the disease. However, in contrast to normal wound repair, it is unclear why in the majority of asthmatics, inflammation leads to ongoing remodelling rather than a self limiting healing process. In this context, cells that line the airways (epithelium) as well as cells that sit immediately beneath them (fibroblasts) are important sources of mediators and structural matrix proteins that contribute to these processes. Under normal conditions, signals from these structural proteins are transmitted to the cells via specific adhesion molecules. However, in asthma epithelial cells are frequently damaged and detached, and fibroblasts appear to proliferate and undergo changes in their appearance. This projects aims to investigate the expression and function of specific cell adhesion molecules in the epithelium and fibroblasts following airway inflammation. Specifically, this proposal aims to determine which adhesion molecules are associated with upregulated proliferation and production of matrix proteins. We will also examine the effects of two novel mediators, thought to play a role in remodelling on the expression and function of these adhesion molecules. Proliferation of these cells and the altered deposition of matrix proteins may be a key feature of airway wall thickening and hyperreactivity that is a characteristic feature of asthma. The balance of deposition and breakdown of matrix proteins is regulated by a variety of mediators. Defining what regulates the expression and activity of adhesion molecules is of fundamental importance in determining how the normal repair processes may evolve into airway wall remodelling.
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    Funded Activity

    Long-lasting Correction Of The Basic Defect In Cystic Fibrosis

    Funder
    National Health and Medical Research Council
    Funding Amount
    $458,500.00
    Summary
    The airway disease caused by the genetic disease cystic fibrosis (CF) is not yet preventable. Current treatments can only limit the gradually-increasing lung disease and is costly. Our new gene therapy technique introduces a correcting gene into affected airway cells, and it has already worked in the first tests in mice bred with CF. Airways in mice are used to test whether the effect is reliable, effective, and lasts long enough to be useful. The gene is introduced into the airway using special .... The airway disease caused by the genetic disease cystic fibrosis (CF) is not yet preventable. Current treatments can only limit the gradually-increasing lung disease and is costly. Our new gene therapy technique introduces a correcting gene into affected airway cells, and it has already worked in the first tests in mice bred with CF. Airways in mice are used to test whether the effect is reliable, effective, and lasts long enough to be useful. The gene is introduced into the airway using special virus delivery-particles, after conditioning the airway to make it receptive to the particles. The method works in normal mice and in CF mice; it gives long lasting gene transfer from a single dose and seems to affect all airway cell types. The gene transfer may also be occurring in airway stem cells, i.e. the mother cells from which grow all the cells of the airway surface. Until now, no-one else has been able to produce prolonged gene transfer in this way, nor arrange gene transfer into stem cells in live airways. There are now a number of things that we must investigate before we could conduct safety and effectiveness trials in larger animals, or consider moving into clinical trials in humans. We need to understand exactly how our conditioning agent works and is it safe; measure how long the gene correction can last actually in our animals; decide if we can we re-dose animals (if needed) without losing effectiveness because of inflammation or immune responses that might occur; and decide how important the airway stem cells are in producing the length of the gene transfer. Because it has been difficult to measure gene correction in CF airways, we will also test new ways we have developed to measure how well the gene correction works in CF airways. The findings of this project will allow us to develop our method to where we can test it in larger animals, to provide a strong, long-lasting gene correction that will be safe for testing in human clinical trials.
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