Dr. David Bedwell
Dr. David Bedwell is the James C. & Elizabeth T. Lee Endowed Chair of Biochemistry and is Professor and Chair of the Department of Biochemistry and Molecular Genetics at the University of Alabama at Birmingham (UAB).
Dr. Bedwell serves as the Co-Director of UAB Structural Biology Program and as Associate Director of the Gregory Fleming James Cystic Fibrosis Research Center. Dr. Bedwell is also an Editorial Board Member for the Journal of Biological Chemistry.
The major goal of Dr. Bedwell’s lab is to develop therapies to treat genetic diseases caused by nonsense mutations. His team uses a combination of genomics, genetics, biochemistry, and cell biology to better understand the molecular details of translation termination in order to develop viable therapeutic strategies aimed at suppressing nonsense mutations and restoring expression of full-length, functional protein.
One disease currently under active investigation is cystic fibrosis (CF). Roughly 10% of CF patients carry a nonsense mutation in the CFTR gene. Dr. Bedwell’s group has participated in the identification of compounds that suppress termination at nonsense mutations in the CFTR gene and are examining these so-called “readthrough” agents in various experimental models. They have also identified the amino acids that become inserted during the suppression of many nonsense mutations in mammalian cells and are using that information to develop strategies to enhance the CFTR activity restored by nonsense suppression using CFTR correctors and potentiators.
Other diseases that Dr. Bedwell’s lab is using to study the general utility of this approach include neurofibromatosis, FOXG1 deficiency, Rett syndrome, and Hurler syndrome. The ultimate goal of this therapeutic approach is to allow the effective treatment of patients with a range of human genetic diseases caused by nonsense mutations