PubMed 19348045

Referenced in Channelpedia wiki pages of: none

Automatically associated channels: Kv1.1 , Kv10.1

Title: The voltage-gated K+ channel subunit Kv1.1 links kidney and brain.

Authors: David H Ellison

Journal, date & volume: J. Clin. Invest., 2009 Apr , 119, 763-6

PubMed link:

Analysis of Mendelian Mg2+ wasting disorders helps us to unravel the mechanisms of Mg2+ homeostasis. In this issue of the JCI, Glaudemans andcolleagues show that mutations in voltage-gated K+ channel subtype 1.1(Kv1.1) cause autosomal dominant hypomagnesemia in humans (see the related article beginning on page 936). Interestingly, other mutations in the same protein cause the neurological disease episodic ataxia type 1. The authors show, using cells with heterologous expression of the wild-type and mutant channels, that the mutant channel is dysfunctional and speculate that Mg2+ wasting results from changes in apical membrane voltage along the nephron. Mechanisms by which the apical voltage is generated and howKv1.1 fits within this context are discussed herein.