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Bear Lab

Cystic Fibrosis Research

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis (CF), the most common fatal congenital disease amongst caucasians. Our work has provided direct evidence that CFTR acts as a phosphorylation and ATP regulated chloride channel and we have characterized the functional properties of the major CF-causing mutations. Currently, we are developing chemical-biology approaches to define the molecular lesion caused by these disease-causing mutations and to identify the molecular targets for therapeutics.

As Co-Director (with Dr. F. Ratjen) of the SickKids Cystic Fibrosis Centre, Dr. Bear is coordinating efforts to discover small molecules and peptides that are effective in correcting the trafficking and functional defects exhibited by CF mutants. Promising “hits” generated through structure-based drug design or high-throughput screens are currently undergoing validation in human respiratory epithelial cultures and in in-vivo studies of transgenic mice, possessing the major CF mutation. Sensitive clinical assays of drug efficacy and safety are being developed and validated to enhance translation of novel therapies.

These CF-related studies are supported by:

The Canadian Cystic Fibrosis Foundation
The Canadian Institutes of Health Research
The Cystic Fibrosis Foundation (US)