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Idiopathic Pulmonary Fibrosis, a Disease Initiated by Mucociliary Dysfunction

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Regents of the University of Colorado Aurora United States

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The overarching goal of this Program is to develop the scientific knowledge needed to predict and prevent the progression of IPF. We postulate that IPF is caused by recurrent injuryrepairregeneration at the bronchoalveolar junction secondary to overexpression of MUC5B, mucociliary dysfunction, retention of particles, ER stress, and disruption of normal reparative and regenerative mechanisms in the distal lung. During the first year of funding, we have 1 obtained local and DoD approvals for human and animal research 2 enrolled 26 first degree relatives of individuals with IPF and completed all study procedures for Project 1 3 performed ChIP, MNase, and TF binding assays to show that MUC5B promoter region is hyperchippable and that HIF1 and GCF bind in this region Project 2 4 imported and bred new strains of mice St3gal3, Fut2, Ern2, Ift88, andArl13b in Projects 3 and 4 5 developed and assessed the amounts and glycosylation of Muc5b in mouse models at baseline, and identified changes in polymer size and migration after inflammatory challenge Project 3.

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Technical Report,30 Sep 2017,29 Sep 2018



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Approved For Public Release;

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