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inwardly-rectifying K+ channel J11; ATP-sensitive inward rectifier K+ channel 11; K+ channel, inwardly rectifying subfamily J member 11; inward rectifier K+ channel Kir6.2; IKATP (KCNJ11)

Function: - inwardly-rectifying K+ channel controlled by G proteins - can be blocked by extracellular Ba+2 (putative) - associates with the sulfonylurea receptor SUR2 & SUR2B - associates with ABCC8/SUR Structure: - belongs to the inward rectifier-type K+ channel family Compartment: membrane Pathology: - defects in KCNJ11 are the cause of a) familial hyperinsulinemic hypoglycemia type 2 b) transient neonatal diabetes mellitus type 3 - defects in KCNJ11 are a cause of - permanent neonatal diabetes mellitus - defects in KCNJ11 may contribute to diabetes mellitus type 2

Related

sulfonylurea receptor 1; ATP-binding cassette transporter sub-family C member 8; ATP-binding cassette C8 (ABCC8, HRINS, SUR SUR1)

General

ATP-sensitive K+ channel (inwardly-rectifying K+ channel) inwardly-rectifying K+ channel subfamily J

Properties

SIZE: entity length = 390 aa MW = 44 kD COMPARTMENT: plasma membrane CELL: beta cell WITHIN: islets of Langerhans ORGANISM: vertebrate* MOTIF: cytoplasmic domain {1-68} transmembrane domain {69-93} exoplasmic loop {94-144} MOTIF: H5 (pore-forming helix) {117-128} peptide motif {130-135} transmembrane domain {145-166} MOTIF: asparagine residue {160} cytoplasmic domain {167-390} ION-PERMEABILITY: K+

Database Correlations

OMIM correlations MORBIDMAP 600937 UniProt Q14654 Pfam PF01007

References

  1. UniProt :accession Q14654
  2. GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/KCNJ11
  3. Liss B & Roeper J Molecular physiology of neuronal K-ATP channels (review). Molecular Membrane Biology 18:117, 2001 PMID: 11463204