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Kir6.2 This receptor is controlled by G proteins. 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. Subunit of ATP-sensitive potassium channels (KATP). Can form cardiac and smooth muscle-type KATP channels with ABCC9. KCNJ11 forms the channel pore while ABCC9 is required for activation and regulation. Defects in KCNJ11 are the cause of familial hyperinsulinemic hypoglycemia type 2 (HHF2); also known as persistent hyperinsulinemic hypoglycemia of infancy (PPHI) or congenital hyperinsulinism. HHF is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. Defects in KCNJ11 are a cause of diabetes mellitus permanent neonatal (PNDM). PNDM is a rare form of diabetes distinct from childhood-onset autoimmune diabetes mellitus type 1. It is characterized by insulin-requiring hyperglycemia that is diagnosed within the first months of life. Permanent neonatal diabetes requires lifelong therapy. Defects in KCNJ11 are the cause of transient neonatal diabetes mellitus type 3 (TNDM3). Neonatal diabetes mellitus, defined as insulin-requiring hyperglycemia within the first month of life, is a rare entity. In about half of the neonates, diabetes is transient and resolves at a median age of 3 months, whereas the rest have a permanent form of diabetes. In a significant number of patients with transient neonatal diabetes mellitus, diabetes type 2 appears later in life. The onset and severity of TNDM3 is variable with childhood-onset diabetes, gestational diabetes or adult-onset diabetes described. Defects in KCNJ11 may contribute to non-insulin- dependent diabetes mellitus (NIDDM), also known as diabetes mellitus type 2. Belongs to the inward rectifier-type potassium channel (TC 1.A.2.1) family. KCNJ11 subfamily. Note: This description may include information from UniProtKB.
Protein type: Channel, potassium; Membrane protein, integral; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 11p15.1
Cellular Component: acrosomal vesicle; ATP-sensitive potassium channel complex; axolemma; cell soma; cytosol; endoplasmic reticulum; endosome; integral component of plasma membrane; mitochondrion; myelin sheath; nuclear envelope; plasma membrane; T-tubule; voltage-gated potassium channel complex
Molecular Function: ankyrin binding; ATP binding; ATP-activated inward rectifier potassium channel activity; heat shock protein binding; potassium ion binding; protein binding; protein C-terminus binding; voltage-gated potassium channel activity
Biological Process: glucose metabolic process; negative regulation of insulin secretion; neurological system process; potassium ion import; regulation of insulin secretion; regulation of membrane potential; response to ATP; response to drug; response to estradiol; response to testosterone stimulus; transmembrane transport
Disease: Diabetes Mellitus, Noninsulin-dependent; Diabetes Mellitus, Permanent Neonatal; Diabetes Mellitus, Transient Neonatal, 3; Hyperinsulinemic Hypoglycemia, Familial, 2; Maturity-onset Diabetes Of The Young, Type 13
Reference #:  Q14654 (UniProtKB)
Alt. Names/Synonyms: ATP-sensitive inward rectifier potassium channel 11; beta-cell inward rectifier subunit; BIR; HHF2; IKATP; Inward rectifier K(+) channel Kir6.2; inwardly rectifying potassium channel KIR6.2; IRK11; KCNJ11; KIR6.2; MGC133230; PHHI; potassium channel inwardly rectifing subfamily J member 11; Potassium channel, inwardly rectifying subfamily J member 11; potassium inwardly-rectifying channel J11; potassium inwardly-rectifying channel, subfamily J, member 11; TNDM3
Gene Symbols: KCNJ11
Molecular weight: 43,541 Da
Basal Isoelectric point: 8.15  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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