Provided is a method for enhancing the activity of a protein, which protein when mutated, folds in an incorrect conformation in the endoplasmic reticulum, by contacting the protein with an effective amount of a compound which specifically binds to the protein and induces the protein to fold into a proper conformation (a specific chaperone). Also provided are methods for enhancing the stability and preventing the degradation of a protein by contacting the protein with an effective amount of a specific chaperone for the protein which stabilizes the protein in a proper conformation.