Genetic or pharmacologic modulation of MUC5B influences the development of pulmonary fibrosis in mice
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Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with a median survival following diagnosis of two to three years and no known curative therapy. A common gain-of-function variant in the promoter of the lung gel-forming mucin gene MUC5B (rs35705950) is the strongest risk factor for the development of IPF, a finding confirmed by 10 independent studies. Based on this observation, I hypothesize that excess expression of MUC5B damages the distal lung, and when combined with an exogenous insult, results in increased fibroproliferation. This project uses three transgenic ...