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URMC / Labs / Sime Lab / Projects / Pulmonary Fibrosis and Lung Scarring

Pulmonary Fibrosis and Lung Scarring

Myofibroblasts

Myofibroblasts stained for α-smooth muscle actin (red) with
immunofluorescence staining.

Lung scarring, or pulmonary fibrosis is a devastating disease with poor prognosis. We are particularly interested in Idiopathic Pulmonary Fibrosis, which is a scarring disease of unknown origin. One of the hallmarks of pulmonary fibrosis is the accumulation of scar forming myofibroblasts, which differentiate from lung fibroblasts during fibrosis. Myofibroblasts secrete extracellular matrix proteins such as collagen, which leads to scarring. Our lab studies the molecular mechanisms surrounding the differentiation of fibroblasts into myofibroblasts. We also study differences in lung fibroblasts that were isolated from patients with Idiopathic Pulmonary Fibrosis compared to fibroblasts isolated from healthy lungs.

Current fibrosis projects in the lab are focused on cellular metabolism and matrix crosslinking in myofibroblasts. In our animal studies, we utilize insults that are known to cause fibrosis, such as ionizing radiation, silica dust, and the chemotherapeutic bleomycin. Techniques and areas of investigation: primary fibroblast isolation from patients, cell culture, preclinical animal models, cell biology, western blot, immunofluorescence and immunohistochemical staining.