Contents

Search

K+ channel subfamily E member 1; K+ voltage-gated channel subfamily E member 1; IKs producing slow voltage-gated K+ channel subunit beta mink; minimal K+ channel; delayed rectifier K+ channel subunit IsK (KCNE1)

Function: - coassembles with voltage-gated K+ channel alpha subunits to modulate gating kinetics - ancillary protein that assembles as a beta subunit with a voltage-gated K+ channel complex of pore-forming alpha subunits - modulates the gating kinetics & enhances stability of the channel complex - assembled with KCNQ1/KVLQT1, forms the slowly activating delayed rectifier cardiac K+ (IKs) channel; the outward current reaches its steady state only after 50 seconds - assembled with KCNH2/HERG, may modulate the rapidly activating component of the delayed rectifying K+ current in heart (IKr); phosphorylation inhibits the K+ current - associates with KCNQ1/KVLQT1 & KCNH2/HERG Structure: belongs to the K+ channel KCNE family Compartment: membrane; single-pass type 1 membrane protein Expression: - expressed in heart, lung, kidney, testis, ovaries, small intestine, peripheral blood leukocytes - not detected in pancreas, spleen, prostate & colon - restrictively localized in the apical membrane portion of epithelial cells Pathology: - defects in KCNE1 are the cause of a) Jervell & Lange-Nielsen syndrome type 2 b) long QT syndrome type 5; c) KCNE1 mutants form channels that open slowly & close rapidly, thereby diminishing K+ currents

Related

voltage-gated K+ channel

General

glycoprotein K+ channel subfamily E phosphoprotein protein subunit

Properties

SIZE: entity length = 129 aa MW = 15 kD COMPARTMENT: cellular membrane MOTIF: N-glycosylation site {N5} N-glycosylation site {N26} transmembrane domain {44-66} Ser phosphorylation site {S102} ION-PERMEABILITY: K+

Database Correlations

OMIM correlations MORBIDMAP 176261 UniProt P15382 Pfam PF02060 Entrez Gene 3753 Kegg hsa:3753

References

  1. UniProt :accession P15382
  2. LQTSdb; Note: KCNE1 mutations page http://www.ssi.dk/graphics/html/lqtsdb/kcne1.htm
  3. genedis; Note: congenital long QT syndrome website http://www.tau.ac.il/lifesci/bioinfo/genedis/heart/long_qt.html
  4. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=KCNE1