Accessory cholera enterotoxin and analogs thereof as activators of calcium dependent chloride channel

摘要

Vibrio cholerae accessory cholera enterotoxin (ACE) activates a calcium dependent chloride channel. Ace and Ace analogs can be administered to patients to treat diseases involving defects in chloride secretion by the cystitis fibrosis transmembrane receptor (CFTR). Cystic fibrosis, Dent's disease, X-linked nephrolithiasis, X-linked recessive hypophosphatemic rickets, autosomal dominant myotonia congenita and autosomal recessive general myotonia can be treated by the administration of Ace or an Ace analog. For cystic fibrosis, administration of Ace or an Ace analog increases chloride secretion in the lungs which increases the amount of airway surface water in the lumen of the lungs.