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J Biol Chem, Vol. 273, Issue 31, 19419-19423, July 31, 1998
From the Department of Cardiovascular Drug Discovery and the
§ Department of Applied Genomics, Bristol-Myers Squibb
Pharmaceutical Research Institute, Princeton, New Jersey 08543-4000
Benign familial neonatal convulsions (BFNC), a
class of idiopathic generalized epilepsy, is an autosomal dominantly
inherited disorder of newborns. BFNC has been linked to mutations in
two putative K+ channel genes, KCNQ2 and
KCNQ3. Amino acid sequence comparison reveals that both
genes share strong homology to KvLQT1, the potassium channel encoded by
KCNQ1, which is responsible for over 50% of inherited long
QT syndrome. Here we describe the cloning, functional expression, and
characterization of K+ channels encoded by
KCNQ2 and KCNQ3 cDNAs. Individually,
expression of KCNQ2 or KCNQ3 in Xenopus oocytes elicits
voltage-gated, rapidly activating K+-selective currents
similar to KCNQ1. However, unlike KCNQ1, KCNQ2 and KCNQ3 currents are
not augmented by coexpression with the KCNQ1
Functional Expression of Two KvLQT1-related Potassium Channels
Responsible for an Inherited Idiopathic Epilepsy
subunit, KCNE1 (minK,
IsK). Northern blot analyses reveal that KCNQ2 and
KCNQ3 exhibit similar expression patterns in different regions within the brain. Interestingly, coexpression of KCNQ2 and
KCNQ3 results in a substantial synergistic increase in current amplitude. Coexpression of KCNE1 with the two channels strongly suppressed current amplitude and slowed kinetics of activation. The
pharmacological and biophysical properties of the K+
currents observed in the coinjected oocytes differ somewhat from those
observed after injecting either KCNQ2 or KCNQ3 by itself. The
functional interaction between KCNQ2 and KCNQ3 provides a framework for
understanding how mutations in either channel can cause a form of
idiopathic generalized epilepsy.
Copyright © 1998 by The American Society for Biochemistry and Molecular Biology, Inc.
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