The Journal of Clinical Investigation | February 1999 | Volume 103 | Number 4 447 It has been nearly 10 years since the identification and cloning of the cystic fibrosis (CF) gene was announced (1). Despite rapid advances in our understanding of the molecular determinants of the disease, CF remains one of the most common lethal inherited autosomal recessive disorders in the Caucasian population...

Cyclic imine sugars were prepared by a novel chemoenzymatic strategy in which azido-sugars, constructed by enzymatic aldol reactions, were hydrogenated under acidic conditions. These cyclic imine sugars were found to be potent inhibitors of glycoprocessing enzymes having Ki’s in the nanomolar and micromolar range for a variety of glycosidases. In comparison with their fully hydrogenated counter...