TITLE: Characterization of mechanisms regulating multiciliated cell specification using patient-specific induced pluripotent stem cells

PROJECT GOAL: The goal of this project is to understand mechanisms of multiciliogenesis and generate a robust iPSC derived in vitro model of multiciliogenesis to evaluate patient specific changes in this pathway

COLLABORATORS: Christopher Kintner (Salk Institute), Zea Borok (UCSD)

 

TITLE: Quantitative analysis of mucociliary clearance in airway ciliopathies

PROJECT GOAL: The goal of this study is to better understand how the spatial organization of the airway epithelium regulates airway clearance in diseases characterized by genetic changes in motile cilia.

COLLABORATORS: Eva Kanso (USC), Janna Nawroth (Helmholtz:Munich)

 

TITLE: Epigenetic dysregulation of airway basal cells by the inflammatory microenvironment

PROJECT GOAL: The long-term goal of this proposal is to generate a long-lived, basal stem cell capable of maintaining function in the microenvironment of the CF lung. To this end, in this proposal, we will investigate how epigenetic changes induced by the hyperinflammatory lung microenvironment, or in response to in vitro culturing, affect maintenance of the MBC in normal lung and are dysregulated in CF lung

COLLABORATORS: Crystal Marconett (USC)

 

TITLE: High throughput screening to maintain pluripotent ferret iPSC for use in pre-clinical autologous cell therapy models

PROJECT GOAL: The goal of this project is to understand the mechanisms that regulate ferret pluripotency toward developing a larger animal model for pre-clinical evaluation of autologous cell therapy.

COLLABORATORS: Xingshen Sun (UIowa)

 

TITLE: Interaction of Lymphatic Endothelial and LAM Cells Driving Lymphangioleiomyomatosis Pathogenesis

PROJECT GOAL: The goal of this funding is to establish a co-culture/organoid model of lymphatic endothelial and LAM cells to understand the role of the lymphatic endothelium in driving LAM pathogenesis.

COLLABORATORS: Sinem Koc-Gunel 

 

TITLE: Targeting the exocrine pancreas to restore function in Cystic Fibrosis-Related Diabetes

PROJECT GOAL: The goal of this project is to generate autologous pancreatic progenitor cells from iPSCs to restore islet function to the CF pancreas after exocrine mediated destruction has occurred. We will define ferret pancreatic development to advance the ferret as a model of CFRD.

COLLABORATORS: Xingshen Sun (UIowa).

 

TITLE: Investigating the role of inflammation in amplifying negative multisystem outcomes from SARS-CoV2 exposure and the pathogenesis of COVID-19

PROJECT GOAL: The goal of this project is to generate autologous pancreatic progenitor cells from iPSCs to restore islet function to the CF pancreas after exocrine mediated destruction has occurred. We will define ferret pancreatic development to advance the ferret as a model of CFRD.

COLLABORATORS: PI - Senta Georgia (USC)

 

TITLE: Repurposing FDA (Food and Drug Administration) approved drugs to improve the standard of care for COVID-19 

PROJECT GOAL: The goal of this project is to determine how regulators of hyaluronan and bradykinin signaling impact airway epithelial cell function and response to SARS-CoV-2 infection. 

COLLABORATORS: None

 

TITLE: RECODE: Defining Environmental Design criteria for Directed Differentiation of Type 1 from Type 2 Lung Alveolar Epithelial Cells

PROJECT GOAL: The goal of this project is to devise and validate a robust system for delineating the mechanisms by which ECM composition and stiffness regulate a key step in lung repair and regeneration: differentiation of alveolar type 2 epithelial cells (AT2s) to alveolar type 1 epithelial cells (AT1s).

COLLABORATORS: Chelsea Magin (U Denver, Colorado: Anschutz), Dan Weiss (UVermont)