ATP-sensitive inward rectifier potassium channel 11

UniProt Q14654 Organism Homo sapiens Gene KCNJ11

Also known as: KCNJ11

Function

Inward rectifier potassium channel that forms the pore of ATP-sensitive potassium channels (KATP), regulating potassium permeability as a function of cytoplasmic ATP and ADP concentrations in many different cells. Inward rectifier potassium channels are characterized by a greater tendency to allow potassium to flow into the cell rather than out of it. Their voltage dependence is regulated by the concentration of extracellular potassium; as external potassium is raised, the voltage range of the channel opening shifts to more positive voltages. The inward rectification is mainly due to the blockage of outward current by internal magnesium. Can be blocked by extracellular barium (By similarity). In pancreatic cells, it forms KATP channels with ABCC8/SUR1. Can form cardiac and smooth muscle-type KATP channels with ABCC9.

Classification

Family (Pfam): PF01007 IRK, PF17655 IRK_C
InterPro: Ig_E-set, IRK_C, K_chnl_inward-rec_Kir, K_chnl_inward-rec_Kir6.2, K_chnl_inward-rec_Kir_cyto, Kir_TM
Functional cluster: Cytochrome P450 & Heme Enzymes

Experimental structures · PDB · 9

6C3O EM 3.90A
6C3P EM 5.60A
7S5T EM 3.10A
7S5X EM 3.70A
7S5Y EM 3.90A
7S5Z EM 3.90A
7S60 EM 3.70A
7S61 EM 4.00A
9KGL EM 2.88A

A predicted model is available from AlphaFold.

Gene Ontology · 22

Disease associations

hyperinsulinemic hypoglycemia, familial, 2 MONDO:0011153
diabetes mellitus, transient neonatal, 3 MONDO:0012522
maturity-onset diabetes of the young type 13 MONDO:0014589
diabetes mellitus, permanent neonatal 2 MONDO:0030087

Drugs targeting this protein · 17

GLIPIZIDE blocker
PINACIDIL opener
TOLBUTAMIDE SODIUM blocker
REPAGLINIDE blocker
GLIMEPIRIDE blocker
ACETOHEXAMIDE blocker
DIAZOXIDE opener
SARAKALIM opener
NAMINIDIL opener
GLICLAZIDE blocker
MITIGLINIDE blocker
GLYBURIDE blocker
CHLORPROPAMIDE blocker
TOLBUTAMIDE blocker
NATEGLINIDE blocker
MINOXIDIL opener
TOLAZAMIDE blocker

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

ATP-binding cassette sub-family C member 8 11 shared papers
ATP-sensitive inward rectifier potassium channel 11 1 shared papers
ATP-sensitive inward rectifier potassium channel 8 1 shared papers
ATP-binding cassette sub-family C member 9 1 shared papers
Apolipoprotein A-IV 1 shared papers
Dopamine beta-hydroxylase 1 shared papers
Thromboxane-A synthase 1 shared papers
Apolipoprotein C-II 1 shared papers
Alpha-adducin 1 shared papers
Apolipoprotein C-I 1 shared papers
Beta-3 adrenergic receptor 1 shared papers
E-selectin 1 shared papers

Literature · 43 cited papers

A loss-of-function mutation in KCNJ11 causing sulfonylurea-sensitive diabetes in early adult life. Diabetologia · 2024
Molecular structure of an open human KATP channel. Proc. Natl. Acad. Sci. U.S.A. · 2021
Molecular structure of human KATP in complex with ATP and ADP. Elife · 2017
Conserved functional consequences of disease-associated mutations in the slide-helix of Kir6.1 and Kir6.2 subunits of the ATP-sensitive potassium channel. J. Biol. Chem. · 2017
Whole-exome sequencing and high throughput genotyping identified KCNJ11 as the thirteenth MODY gene. PLoS ONE · 2012
Interaction between mutations in the slide helix of Kir6.2 associated with neonatal diabetes and neurological symptoms. Hum. Mol. Genet. · 2010
Sar1-GTPase-dependent ER exit of KATP channels revealed by a mutation causing congenital hyperinsulinism. Hum. Mol. Genet. · 2009
Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations. J. Clin. Invest. · 2008
Functional modulation of the ATP-sensitive potassium channel by extracellular signal-regulated kinase-mediated phosphorylation. Neuroscience · 2008
A mutation in the ATP-binding site of the Kir6.2 subunit of the KATP channel alters coupling with the SUR2A subunit. J. Physiol. (Lond.) · 2007
A novel mutation causing DEND syndrome: a treatable channelopathy of pancreas and brain. Neurology · 2007
Prevalence of permanent neonatal diabetes in Slovakia and successful replacement of insulin with sulfonylurea therapy in KCNJ11 and ABCC8 mutation carriers. J. Clin. Endocrinol. Metab. · 2007
… and 31 more in the literature graph

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