Amy Wong

Establishment of iPS Cells from lung Epithelia of Patients with Cystic Fibrosis

Cystic fibrosis (CF) is a genetically fatal disease that affects lung epithelial cells. The current best treatment for patients with CF is lung transplantation. However, the shortage of lung donors and risks of transplant rejection impedes the chances of survival. Furthermore, the limited availability of differentiated patient-specific CF lung epithelium remains a major road-block for the potential development of therapeutic drugs to treat the CF disease.

Induced pluripotent stem (iPS) cells are a novel promising cell type that can be generated from normal or disease-specific human skin cells. Following manipulation, these cells resemble embryonic stem (ES) cells and are capable of generating all cell types. Epithelial cells obtained from this method can then be used to establish high-throughput screening methods for identifying and measuring novel chemical/peptides with therapeutic potential in treating CF.

The goal of my project is to establish a reliable and efficient protocol to generate lung epithelia from CF patient-derived iPS cells (Co-supervisor Dr. James Ellis). To aid in identifying iPS-derived epithelial cell, a set of fluorescent reporter genes to identify and select the cell of interest will be developed in collaboration with Dr. Jim Hu (The Hospital for Sick Children, Toronto).

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