An increasing number of genetic diseases are found to result from mutations that lead to
retention of the affected proteins in the endoplasmic reticulum, where they are recognized as
misfolded by the quality control system. Several of these conformational diseases involve
mutations in G-protein-coupled receptors. Recent studies demonstrated that
pharmacologically selective compounds, termed pharmacological chaperones, can stabilize
the misfolded receptors, facilitating their export from the endoplasmic reticulum to the …