Welcome to the Christine Bear Laboratory website. Dr. Bear’s laboratory group studies the role of membrane proteins in human diseases affecting the respiratory and renal systems. Our goal is to understand the mechanism of action of chloride channels and transporters implicated in human disease.
We study cystic fibrosis (CF), a disease that affects the lungs, digestive, and reproductive organs. CF is caused by defects in the cystic fibrosis transmembrane conductance regulator (CFTR), which is a membrane protein that normally mediates chloride conductance and drives epithelial transport in the previously mentioned organs. Our work focuses on understanding the function of the normal CFTR protein, the molecular defects caused by the major CF mutant proteins, and defining novel therapeutic strategies by which to repair these defects.
We also study function of the proximal tubule of the kidney – a major site for the regulation of urinary protein loss. Mutations in the transporter protein ClC-5 are linked to proteinuria, renal stone formation, and renal failure in Dent’s disease. Currently, we are working to understand the cellular and molecular basis for this disease.
We have published extensively in these areas in prestigious publications such as Cell, Nature Genetics and EMBO Journal, and the studies were supported by several funding agencies, including the CIHR, NIH (USA), Canadian Cystic Fibrosis Foundation (CCFF), the US-CFF and the Kidney Foundation of Canada.
As Co-Director of the SickKids Cystic Fibrosis Centre, Dr. Bear is coordinating the efforts of basic and clinical researchers for the development of small molecule therapies for correction of the primary CF defect.