Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia with a median survival time of 2-4 years after diagnosis. The alarming mortality rate is due to the lack of effective treatments. IPF is a chronic disease that is characterized by alveolar destruction due to increasing extracellular matrix deposition that leads to poor lung compliance, impaired gas exchange, and ultimately respiratory failure. Repetitive alveolar epithelial injury is a central process to the underlying pathology with injury to the type II alveolar epithelial cells (AT2) specifically being a key player in the pathogenesis of IPF. Recent studies have shown that recapitulation of developmental genes in AT2 cells is associated with the abnormal epithelial phenotype seen in IPF, however the specific genes and the mechanisms of how they alter epithelial function are poorly understood. The work in this dissertation addresses one such developmental gene, Sine Oculis Homeobox Homolog 1 (Six1), which is a transcription factor that is essential for normal lung morphogenesis in utero that I show to be inappropriately expressed in the AT2 cells in IPF.
","abstract_html":"<p>Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia with a median survival time of 2-4 years after diagnosis. The alarming mortality rate is due to the lack of effective treatments. IPF is a chronic disease that is characterized by alveolar destruction due to increasing extracellular matrix deposition that leads to poor lung compliance, impaired gas exchange, and ultimately respiratory failure. Repetitive alveolar epithelial injury is a central process to the underlying pathology with injury to the type II alveolar epithelial cells (AT2) specifically being a key player in the pathogenesis of IPF. Recent studies have shown that recapitulation of developmental genes in AT2 cells is associated with the abnormal epithelial phenotype seen in IPF, however the specific genes and<em> </em>the mechanisms of how they alter epithelial function are poorly understood. The work in this dissertation addresses one such developmental gene, Sine Oculis Homeobox Homolog 1 (Six1), which is a transcription factor that is essential for normal lung morphogenesis <em>in utero</em> that I show to be inappropriately expressed in the AT2 cells in IPF.</p>","abstract_has_math":false,"creators":["Wilson, Cory",""],"institution":null,"degree_name":"Doctor of Philosophy (PhD)","degree_level":"Dissertation (PhD)","degree_discipline":null,"degree_department":null,"school":null,"contributors":["Harry Karmouty-Quintana, Ph.D.","Holger Eltzschig, M.D. Ph.D.","Shane Cunha, Ph.D."],"advisors":[],"committee_chairs":[],"committee_members":[],"year":2020,"date_issued":"2020-12-01T08:00:00Z","date_published":"2020-12-01T08:00:00Z","updated_at":"2026-07-24T05:50:16Z","subjects":["Idiopathic Pulmonary Fibrosis","Interstitial pulmonary disease","Six1","Eya1","Eya2","MIF","alveolar type II epithelial cells","AT2","IPF","lung fibrosis","Biochemistry","Cell Biology","Integrative Biology","Medicine and Health Sciences","Molecular Biology"],"languages":[],"rights":[],"rights_urls":[],"identifier_entries":[]},"links":{"outbound_url":"https://digitalcommons.library.tmc.edu/utgsbs_dissertations/1056","outbound_label":"Repository record","outbound_source":"dc:identifier"},"metadata_groups":[{"id":"people","label":"People","entries":[{"key":"dc:contributor","label":"Contributor","values":["Harry Karmouty-Quintana, Ph.D.","Holger Eltzschig, M.D. Ph.D.","Shane Cunha, Ph.D."]},{"key":"dc:creator","label":"Author","values":["Wilson, Cory",""]}]},{"id":"academic_context","label":"Academic Context","entries":[{"key":"dc:date.available","label":"Dc Date Available","values":["2021-12-16T08:00:00Z"]},{"key":"thesis:degree_level","label":"Degree Level","values":["Dissertation (PhD)"]},{"key":"thesis:degree_name","label":"Degree Name","values":["Doctor of Philosophy (PhD)"]}]},{"id":"subjects_keywords","label":"Subjects and Keywords","entries":[{"key":"dc:subject","label":"Dc Subject","values":["Idiopathic Pulmonary Fibrosis","Interstitial pulmonary disease","Six1","Eya1","Eya2","MIF","alveolar type II epithelial cells","AT2","IPF","lung fibrosis","Biochemistry","Cell Biology","Integrative Biology","Medicine and Health Sciences","Molecular Biology"]}]},{"id":"identifiers","label":"Identifiers","entries":[{"key":"dc:identifier","label":"Identifier","values":["https://digitalcommons.library.tmc.edu/utgsbs_dissertations/1056"]}]},{"id":"additional","label":"Additional Metadata","entries":[{"key":"dc:description.abstract","label":"Abstract","values":["Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia with a median survival time of 2-4 years after diagnosis. The alarming mortality rate is due to the lack of effective treatments. IPF is a chronic disease that is characterized by alveolar destruction due to increasing extracellular matrix deposition that leads to poor lung compliance, impaired gas exchange, and ultimately respiratory failure. Repetitive alveolar epithelial injury is a central process to the underlying pathology with injury to the type II alveolar epithelial cells (AT2) specifically being a key player in the pathogenesis of IPF. Recent studies have shown that recapitulation of developmental genes in AT2 cells is associated with the abnormal epithelial phenotype seen in IPF, however the specific genes and the mechanisms of how they alter epithelial function are poorly understood. The work in this dissertation addresses one such developmental gene, Sine Oculis Homeobox Homolog 1 (Six1), which is a transcription factor that is essential for normal lung morphogenesis in utero that I show to be inappropriately expressed in the AT2 cells in IPF.
"]},{"key":"dc:title","label":"Title","values":["Sine Oculis Homeobox Homolog 1 (Six1) Plays A Critical Role In The Progression of Pulmonary Fibrosis."]}]}],"canonical_facts":{"dc:contributor":["Harry Karmouty-Quintana, Ph.D.","Holger Eltzschig, M.D. Ph.D.","Shane Cunha, Ph.D."],"dc:creator":["Wilson, Cory",""],"dc:date.available":["2021-12-16T08:00:00Z"],"dc:description.abstract":["Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia with a median survival time of 2-4 years after diagnosis. The alarming mortality rate is due to the lack of effective treatments. IPF is a chronic disease that is characterized by alveolar destruction due to increasing extracellular matrix deposition that leads to poor lung compliance, impaired gas exchange, and ultimately respiratory failure. Repetitive alveolar epithelial injury is a central process to the underlying pathology with injury to the type II alveolar epithelial cells (AT2) specifically being a key player in the pathogenesis of IPF. Recent studies have shown that recapitulation of developmental genes in AT2 cells is associated with the abnormal epithelial phenotype seen in IPF, however the specific genes and the mechanisms of how they alter epithelial function are poorly understood. The work in this dissertation addresses one such developmental gene, Sine Oculis Homeobox Homolog 1 (Six1), which is a transcription factor that is essential for normal lung morphogenesis in utero that I show to be inappropriately expressed in the AT2 cells in IPF.
"],"dc:identifier":["https://digitalcommons.library.tmc.edu/utgsbs_dissertations/1056"],"dc:subject":["Idiopathic Pulmonary Fibrosis","Interstitial pulmonary disease","Six1","Eya1","Eya2","MIF","alveolar type II epithelial cells","AT2","IPF","lung fibrosis","Biochemistry","Cell Biology","Integrative Biology","Medicine and Health Sciences","Molecular Biology"],"dc:title":["Sine Oculis Homeobox Homolog 1 (Six1) Plays A Critical Role In The Progression of Pulmonary Fibrosis."],"thesis:degree_level":["Dissertation (PhD)"],"thesis:degree_name":["Doctor of Philosophy (PhD)"]},"updated_at":"2026-07-24T05:50:16Z"}