Understanding basal cell niche and cellular heterogeneity in CF

Project: Research project

Project Details


Understanding basal cell niche and cellular heterogeneity in CF Understanding basal cell niche and cellular heterogeneity in CF Cystic Fibrosis (CF) is the most common life threatening genetic lung disease with no effective treatment. Currently we are only able to provide symptomatic relief for patients. In the past decade technologies that allow us to take a patients skin cells and reprogram them into a primitive stem cell that has the capacity to then become any cell in the body, known as induced pluripotent stem cells or iPSC, has revolutionized our access to human biology. These cells could provide an unlimited supply of airway epithelial cells with the potential to engraft and repair a damaged CF lung. Unfortunately, the precise identity of the mot regenerative cells in the lungs remains poorly defined. In addition, significant change inside the lung such as increased inflammation; epithelial injury and tissue remodeling are all critical components of disease progression and may alter the potential of these cells to repair the injured lung. We propose in our study to evaluate differences in cellular identity, signaling and differentiation capacity in iPSC- derived lung stem cells, known as basal cells comparing 1) CF and non-CF patient iPSC and 2) patient specific mutant and gene- corrected iPSC-derived basal cells. Ultimately, we hope to identify cellular signaling molecules that can be used to optimize the generation of basal cells from iPSC for cell therapy for CF patients or that can stimulate cellular restoration in the injured CF lung.
Effective start/end date2/1/181/31/20


  • Cystic Fibrosis Foundation: $40,000.00


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