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Previous Volume 336:1575-1586 May 29, 1997 Number 22 Next

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Ion channels constitute a class of proteins that is ultimately responsible for generating and orchestrating the electrical signals passing through the thinking brain, the beating heart, and the contracting muscle. Using the methods of molecular biology and patch-clamp electrophysiology, investigators have recently cloned, expressed, and characterized the genes encoding many of these proteins. Ion-channel proteins are under intense scrutiny in an effort to determine their roles in pathophysiology and as potential targets for drugs.

Defective ion-channel proteins are responsible for cystic fibrosis,¹ the long-QT syndrome,² heritable hypertension (Liddle's syndrome),^3,4 familial persistent hyperinsulinemic hypoglycemia of infancy,^5,6 hereditary nephrolithiasis (Dent's disease), and . . . [Full Text of this Article]

Physiology of Ion Channels

Molecular Blueprints of Ion Channels

Heritable Diseases Associated with Ion-Channel Mutations

Cystic Fibrosis

Long-QT Syndrome

Targeting Ion Channels

The ATP-Sensitive Potassium Channel

The G-Protein–Activated Potassium Channel

Conclusions

Source Information

From the Department of Pediatrics and Adolescent Medicine, Mayo Foundation, Rochester, Minn. (M.J.A.); and the Department of Cardiology, Children's Hospital Medical Center, Department of Neurobiology, Harvard Medical School, Boston (D.E.C.).

Address reprint requests to Dr. Ackerman at the Department of Pediatrics and Adolescent Medicine, Mayo Eugenio Litta Children's Hospital, Mayo Foundation, Rochester, MN 55905.

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