K+ voltage-gated channel subfamily E member 3; minK-related peptide 2; minimum K+ channel-related peptide 2; K+ channel subunit beta MiRP2 (KCNE3)

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
• association with KCNC4/Kv3.4 is proposed to form the subthreshold voltage-gated K+ channel in skeletal muscle & to establish the resting membrane potential in muscle cells
• association with KCNQ1/KCLQT1 may form the intestinal cAMP-stimulated K+ channel involved in chloride secretion

Structure

• belongs to the K+ channel KCNE family

Compartment

• membrane; single-pass type 1 membrane protein

Expression

• widely expressed
• highest levels in kidney
• moderate levels in small intestine

Pathology

• defects in KCNE3 are a cause of
  • hypokalemic periodic paralysis
  • susceptibility to thyrotoxic periodic paralysis type 1
• defects in KCNE3 are the cause of Brugada syndrome type 6

More general terms

• glycoprotein
• K+ channel subfamily E

Additional terms

• familial periodic paralysis (hyperkalemic, normokalemic, hypokalemic, HYPP, NKPP, HYPOPP)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=10008
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:10008
• OMIM: https://mirror.omim.org/entry/170400
• OMIM: https://mirror.omim.org/entry/188580
• OMIM: https://mirror.omim.org/entry/604433
• OMIM: https://mirror.omim.org/entry/613119
• UniProt: http://www.uniprot.org/uniprot/Q9Y6H6.html