Hosted by the Department of Biochemistry
Mandatory for all Biochemistry Graduate Students
David Thomas, PhD FRSC
Department of Biochemistry
Cells have homeostatic mechanisms that monitor the integrity organelles and proteins and repair or degrade faulty components, termed proteostasis. One example that we have intensively studied is the ER quality system, the Calnexin Cycle. Protein trafficking diseases such as cystic fibrosis arise from recognition of mutant proteins by the quality control mechanism and the retention of the mutant but otherwise function protein in the ER. We have identified correctors of the trafficking of ER retained mutant proteins and identified two types of mechanism, pharmacological chaperones that bind the mutant molecules and proteostasis modulators that act on the quality control mechanisms.