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Vol. 96, Issue 9, 4759-4766, April 27, 1999

Review
 Ion channel genes and human neurological disease: Recent progress, prospects, and challenges

Edward C. Cooper* and Lily Yeh Jandagger ,Dagger

Departments of * Neurology, and dagger  Physiology, Biochemistry, and Howard Hughes Medical Institute, University of California, San Francisco, CA 94143

Contributed by Lily Yeh Jan, December 31, 1998

What do epilepsy, migraine headache, deafness, episodic ataxia, periodic paralysis, malignant hyperthermia, and generalized myotonia have in common? These human neurological disorders can be caused by mutations in genes for ion channels. Many of the channel diseases are "paroxysmal disorders" whose principal symptoms occur intermittently in individuals who otherwise may be healthy and active. Some of the ion channels that cause human neurological disease are old acquaintances previously cloned and extensively studied by channel specialists. In other cases, however, disease-gene hunts have led the way to the identification of new channel genes. Progress in the study of ion channels has made it possible to analyze the effects of human neurological disease-causing channel mutations at the level of the single channel, the subcellular domain, the neuronal network, and the behaving organism.

Dagger    To whom reprint requests should be addressed at: Box 0725, Howard Hughes Medical Institute, University of California, San Francisco, CA 94143. e-mail: gkw{at}itsa.ucsf.edu.

Copyright © 1999 by The National Academy of Sciences  0027-8424/99/964759-8$2.00/0
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