Fibrosis of the Airway and Lung Tissue
Fibrosis is the abnormal scarring of normal tissue that leads to loss of tissue and organ function. At the present time there are no treatments for fibrotic disorders besides transplant. Fibrotic scarring can occur in the airways or in the lung tissue. The severity of the fibrosis can range from mild which is often asymptomatic to severe with is life limiting. There are over 140 known causes of pulmonary fibrosis. The common theme for these causes is that they create a chronic injury in the lung tissue that leads to the scarring reaction. Pulmonary fibrosis is a common complication of radiation or chemotherapy for cancers and limits the amounts of radiation or drugs that can be administered. In some cases the cause of is unknown (idiopathic).
LRRI is developing animal models of fibrosis to study the etiology of the disorders and for the evaluation of potential therapeutics which could either prevent the fibrosis or slow its progression. At the present time, certain forms pulmonary fibrotic disorders such as idiopathic pulmonary fibrosis are detected too late for any likely therapeutic intervention. These individuals often have a prognosis similar to that of lung cancer patients with mean survival times of 12-24 months post initial diagnosis. However, other forms of pulmonary fibrosis such as those induced by adverse chemotherapeutic drug reactions, radiation treatment or exposure to chemical weapon agents such as sulfur mustard could be responsive to appropriate therapies as treatment could begin before the fibrotic mechanisms have had sufficient time to become refractory to drug treatment.
Contract research services include animal models of drug, radiation and chemical induced forms of lung fibrosis. Both in vitro and in vivo testing of anti-fibrotic therapeutics are available.
Current Research Interests
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Radiation induced pulmonary fibrosis.
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Bleomycin induced pulmonary fibrosis, with an improved aerosol delivery model of bleomycin.
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Sulfur mustard induced lung fibrosis and scarring.
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Immune mediated fibrosis as a result of transplant.
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Regulatory roles of TGF-? and CTGF in fibrotic scarring.
Experimental Capabilities
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Radiation, bleomycin and Sulfur mustard injury models in animals for studies of fibrotic mechanisms and therapeutic validation.
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In vitro models for therapeutic evaluation of anti-fibrotic therapeutics.
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Tracheal transplant model of allogenic versus syngenic grafts.
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Cell and Molecular Biological, Proteomic and Immunologic analysis of cytokines, matrix proteins and other markers of fibrotic scarring.
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Sophisticated histopathology, morphometry, immunohistochemistry and in situ hybridization for analysis of lung tissues.