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M109325200v1
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Papers In Press, published online ahead of print October 25, 2001
J. Biol. Chem, 10.1074/jbc.M109325200
Submitted on September 26, 2001
Revised on October 25, 2001
Accepted on October 25, 2001

Episodic ataxia type-1 mutations in the Kv1.1 potassium channel display distinct folding and intracellular trafficking properties

Louis N. Manganas, Sobia Akhtar, Dana E. Antonucci, Claire R. Campomanes, J. Oliver Dolly, and James S. Trimmer

Department of Molecular and Cellular Physiology, Stanford School of Medicine, Stanford, CA 94305-5345

Corresponding Author: james.trimmer{at}sunysb.edu

Episodic ataxia type 1 (EA-1) is a neurological disorder arising from mutations in the Kv1.1 potassium channel alpha subunit. EA-1 patients exhibit substantial phenotypic variability resulting from at least fourteen distinct EA-1 point mutations. We found that EA-1 missense mutations generate mutant Kv1.1 subunits with folding and intracellular trafficking properties indistinguishable from wild-type Kv1.1. However, the single identified EA-1 nonsense mutation exhibits intracellular aggregation and detergent insolubility. This phenotype can be transferred to co-assembled Kv1 alpha and Kvbeta subunits associated with Kv1.1 in neurons. These results suggest that as in many neurodegenerative disorders, intracellular aggregation of misfolded Kv1.1-containing channels may contribute to the pathophysiology of EA-1.


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