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Title: Kcne3  
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Potassium voltage-gated channel, Isk-related family, member 3
Symbols  ; HOKPP; HYPP; MiRP2
External IDs GeneCards:
RNA expression pattern
Species Human Mouse
RefSeq (mRNA)
RefSeq (protein)
Location (UCSC)
PubMed search

Potassium voltage-gated channel, Isk-related family, member 3, also known as KCNE3, is a protein that in humans is encoded by the KCNE3 gene.[1][2]


  • Function 1
  • See also 2
  • References 3
  • Further reading 4
  • External links 5


Voltage-gated potassium channels (Kv) represent the most complex class of voltage-gated ion channels from both functional and structural standpoints. Their diverse functions include regulating neurotransmitter release, heart rate, insulin secretion, neuronal excitability, epithelial electrolyte transport, smooth muscle contraction, and cell volume. This gene encodes a member of the potassium channel, voltage-gated, isk-related subfamily. This member is a type I membrane protein, and a beta subunit that assembles with a potassium channel alpha-subunit to modulate the gating kinetics and enhance stability of the multimeric complex. This gene is prominently expressed in the kidney. Mutations in this gene are associated with hypokalemic periodic paralysis[1] and Brugada syndrome.[3] KCNE3 is thought to be an accessory protein that serves to inhibit the fast inactivating Kv channel Kv4.3 (the A-current).[4]

See also


  1. ^ a b "Entrez Gene: KCNE3 potassium voltage-gated channel, Isk-related family, member 3". 
  2. ^ Abbott GW, Sesti F, Splawski I, Buck ME, Lehmann MH, Timothy KW, Keating MT, Goldstein SA (April 1999). "MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmia". Cell 97 (2): 175–87.  
  3. ^ Delpón E, Cordeiro JM, Núñez L, Thomsen PE, Guerchicoff A, Pollevick GD, Wu Y, Kanters JK, Larsen CT, Burashnikov E, Christiansen M, Antzelevitch C (2008). "Functional Effects of KCNE3 Mutation and its Role in the Development of Brugada Syndrome". Circ Arrhythm Electrophysiol 1 (3): 209–218.  
  4. ^ Lundby A, Olesen, SP (2006). "KCNE3 is an inhibitory subunit of the Kv4.3 potassium channel". Biochemical and Biophysical Research Communications 346 (3): 958–967.  

Further reading

  • Bonaldo MF, Lennon G, Soares MB (1997). "Normalization and subtraction: two approaches to facilitate gene discovery". Genome Res. 6 (9): 791–806.  
  • Schroeder BC, Waldegger S, Fehr S et al. (2000). "A constitutively open potassium channel formed by KCNQ1 and KCNE3". Nature 403 (6766): 196–9.  
  • Melman YF, Domènech A, de la Luna S, McDonald TV (2001). "Structural determinants of KvLQT1 control by the KCNE family of proteins". J. Biol. Chem. 276 (9): 6439–44.  
  • Abbott GW, Butler MH, Bendahhou S et al. (2001). "MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysis". Cell 104 (2): 217–31.  
  • Dedek K, Waldegger S (2002). "Colocalization of KCNQ1/KCNE channel subunits in the mouse gastrointestinal tract". Pflugers Arch. 442 (6): 896–902.  
  • Abbott GW, Goldstein SA (2002). "Disease-associated mutations in KCNE potassium channel subunits (MiRPs) reveal promiscuous disruption of multiple currents and conservation of mechanism". FASEB J. 16 (3): 390–400.  
  • Mazhari R, Nuss HB, Armoundas AA et al. (2002). "Ectopic expression of KCNE3 accelerates cardiac repolarization and abbreviates the QT interval". J. Clin. Invest. 109 (8): 1083–90.  
  • Dias Da Silva MR, Cerutti JM, Arnaldi LA, Maciel RM (2002). "A mutation in the KCNE3 potassium channel gene is associated with susceptibility to thyrotoxic hypokalemic periodic paralysis". J. Clin. Endocrinol. Metab. 87 (11): 4881–4.  
  • Strausberg RL, Feingold EA, Grouse LH et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903.  
  • Jurkat-Rott K, Lehmann-Horn F (2004). "Periodic paralysis mutation MiRP2-R83H in controls: Interpretations and general recommendation". Neurology 62 (6): 1012–5.  
  • Gerhard DS, Wagner L, Feingold EA et al. (2004). "The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7.  
  • Lundquist AL, Turner CL, Ballester LY, George AL (2006). "Expression and transcriptional control of human KCNE genes". Genomics 87 (1): 119–28.  
  • Panaghie G, Tai KK, Abbott GW (2006). "Interaction of KCNE subunits with the KCNQ1 K+ channel pore". J. Physiol. (Lond.) 570 (Pt 3): 455–67.  
  • Oh JH, Yang JO, Hahn Y et al. (2006). "Transcriptome analysis of human gastric cancer". Mamm. Genome 16 (12): 942–54.  
  • Doi K, Sato T, Kuramasu T et al. (2006). "Ménière's disease is associated with single nucleotide polymorphisms in the human potassium channel genes, KCNE1 and KCNE3". ORL J. Otorhinolaryngol. Relat. Spec. 67 (5): 289–93.  
  • Abbott GW, Butler MH, Goldstein SA (2006). "Phosphorylation and protonation of neighboring MiRP2 sites: function and pathophysiology of MiRP2-Kv3.4 potassium channels in periodic paralysis". FASEB J. 20 (2): 293–301.  
  • Pannaccione A, Boscia F, Scorziello A et al. (2007). "Up-regulation and increased activity of KV3.4 channels and their accessory subunit MinK-related peptide 2 induced by amyloid peptide are involved in apoptotic neuronal death". Mol. Pharmacol. 72 (3): 665–73.  

External links

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