Search
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
a) hypokalemic periodic paralysis
b) susceptibility to thyrotoxic periodic paralysis type 1
- defects in KCNE3 are the cause of Brugada syndrome type 6
Related
familial periodic paralysis (hyperkalemic, normokalemic, hypokalemic, HYPP, NKPP, HYPOPP)
General
glycoprotein
K+ channel subfamily E
Properties
SIZE: entity length = 103 aa
MW = 12 kD
COMPARTMENT: cellular membrane
MOTIF: N-glycosylation site {N5}
N-glycosylation site {N22}
N-glycosylation site {N41}
transmembrane domain {58-78}
ION-PERMEABILITY: K+
Database Correlations
OMIM correlations
MORBIDMAP 604433
UniProt Q9Y6H6
Pfam PF02060
Entrez Gene 10008
Kegg hsa:10008
References
UniProt :accession Q9Y6H6