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K+ voltage-gated channel subfamily KQT member 1; voltage-gated K+ channel subunit Kv7.1; IKs producing slow voltage-gated K+ channel subunit alpha KvLQT1; KQT-like 1 (KCNQ1, KCNA8, KCNA9, KVLQT1, KCNQ1OT1)

Function: - probably important in cardiac repolarization - associates with KCNE1 (minK) to form the I(Ks) cardiac K+ current - elicits a rapidly activating, K+ selective outward current - muscarinic agonist oxotremorine-M strongly suppresses KCNQ1/KCNE1 current in CHO cells in which cloned KCNQ1/KCNE1 channels were coexpressed with M1 muscarinic receptors - may associate also with KCNE3 (MiRP2) to form the K+ channel that is important for cyclic AMP-stimulated intestinal secretion of chloride ions (reduced in cystic fibrosis & pathologically stimulated in cholera & other forms of secretory diarrhea) - heterotetramer with KCNE1 (minK) or KCNE3 (MiRP2) - interacts with CALM Structure: - the segment S4 is probably the voltage-sensor & is characterized by a series of positively charged amino acids at every third position - belongs to the K+ channel family, KQT subfamily Compartment: - cell membrane; multi-pass membrane protein - cytoplasmic vesicle membrane Alternative splicing: named isoforms=2; additional isoforms seem to exist; truncated isoform non-functional alone but modulatory when coexpressed with the full-length isoform 1 Expression: - abundantly expressed in heart, pancreas, prostate, kidney, small intestine & peripheral blood leukocytes - less abundant in placenta, lung, spleen, colon, thymus, testis & ovaries Pathology: - defects in KCNQ1 are the cause of a) long QT syndrome type 1 b) Jervell & Lange-Nielsen syndrome type-1 c) atrial fibrillation familial type 3 d) short QT syndrome type 2

Related

KCNQ1 gene mutation; KCNQ1OT1 gene mutation KVLQT1 gene

General

voltage-gated K+ channel

Properties

SIZE: entity length = 676 aa MW = 75 kD COMPARTMENT: cytoplasm MOTIF: transmembrane domain {122-142} transmembrane domain {148-168} transmembrane domain {197-217} transmembrane domain {226-248} transmembrane domain {262-282} N-glycosylation site {N289} Segment H5 (pore-forming) {300-320} MOTIF: peptide motif {312-317} transmembrane domain {328-348} Subunits assembly {589-620} ION-PERMEABILITY: K+ SUBUNITS: voltage-gated K+ channel alpha subunit (4) MOTIF: transmembrane domain (6) H5 (K+) pore-forming region ION-PERMEABILITY: K+ voltage-gated K+ channel beta subunit MOTIF: phosphorylation site GENE: KVLQT1 gene

Database Correlations

OMIM correlations MORBIDMAP 607542 UniProt P51787 PFAM correlations Entrez Gene 3784 Kegg hsa:3784

References

  1. UniProt :accession P51787
  2. LQTSdb; Note: KCNQ1 mutations page http://www.ssi.dk/en/forskning/lqtsdb/kvlqt1.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=KCNQ1
  5. Wikipedia; Note: KvLQT1 entry http://en.wikipedia.org/wiki/KvLQT1

Components

voltage-gated K+ channel alpha subunit voltage-gated K+ channel beta subunit