K+ voltage-gated channel subfamily E member 2; minK-related peptide 1; minimum K+ channel-related peptide 1; K+ channel subunit beta MiRP1 (KCNE2)
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Function
- 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
- associated with KCNH2/HERG
- proposed to form the rapidly activating component of the delayed rectifying K+ current in heart (IKr)
- may associate with KCNQ2 &/or KCNQ3 & modulate the native M-type current
- may associate with KCNQ1/KVLTQ1 & elicit a voltage-independent current
- may associate with HCN1 & HCN2 & increase K+ current
- associates with KCNH2/ERG1
- may associate with KCNQ1/KVLQT1, KCNQ2 & KCNQ3
- associates with HCN1 & probably HCN2 (putative)
Structure
belongs to the K+ channel KCNE family
Compartment
- membrane; single-pass type 1 membrane protein
Expression
- highly expressed in brain, heart, skeletal muscle, pancreas, placenta, kidney, colon & thymus
- low but significant expression in liver, ovary, testis, prostate, small intestine & leukocytes
- very low expression, nearly undetectable, in lung & spleen
Pathology
- defects in KCNE2 are the cause of
- long QT syndrome type 6
- atrial fibrillation familial type 4
More general terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/Q9Y6J6.html
- ↑ LQTSdb; Note: KCNE2 mutations page http://Www.ssi.dk/en/forskning/lqtsdb/kcne2.htm
- ↑ GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/KCNE2