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)
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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
- Jervell & Lange-Nielsen syndrome type 2
- long QT syndrome type 5;
- KCNE1 mutants form channels that open slowly & close rapidly, thereby diminishing K+ currents
More general terms
Additional terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/P15382.html
- ↑ LQTSdb; Note: KCNE1 mutations page http://www.ssi.dk/graphics/html/lqtsdb/kcne1.htm
- ↑ genedis; Note: congenital long QT syndrome website http://www.tau.ac.il/lifesci/bioinfo/genedis/heart/long_qt.html
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=KCNE1