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K+ voltage-gated channel subfamily E member 2; minK-related peptide 1; minimum K+ channel-related peptide 1; K+ channel subunit beta MiRP1 (KCNE2)

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 a) long QT syndrome type 6 b) atrial fibrillation familial type 4

General

glycoprotein K+ channel subfamily E phosphoprotein

Properties

SIZE: entity length = 123 aa MW = 14 kD COMPARTMENT: cellular membrane MOTIF: N-glycosylation site {N6} N-glycosylation site {N29} Tyr phosphorylation site {Y47} Tyr phosphorylation site {Y48} transmembrane domain {49-69} ION-PERMEABILITY: K+

Database Correlations

OMIM correlations MORBIDMAP 603796 UniProt Q9Y6J6 Pfam PF02060 Entrez Gene 9992 Kegg hsa:9992

References

  1. UniProt :accession Q9Y6J6
  2. LQTSdb; Note: KCNE2 mutations page http://Www.ssi.dk/en/forskning/lqtsdb/kcne2.htm
  3. GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/KCNE2