Gene Symbol: Kcnj11
Description: potassium voltage-gated channel subfamily J member 11
Alias: Kir6.2, ATP-sensitive inward rectifier potassium channel 11, BIR, inward rectifier K(+) channel Kir6.2, potassium channel, inwardly rectifying subfamily J member 11, potassium inwardly rectifying channel, subfamily J, member 11
Species: rat
Products:     Kcnj11

Top Publications

  1. Pratt E, Yan F, Gay J, Stanley C, Shyng S. Sulfonylurea receptor 1 mutations that cause opposite insulin secretion defects with chemical chaperone exposure. J Biol Chem. 2009;284:7951-9 pubmed publisher
    ..Our results show that sulfonylureas, as chemical chaperones, can dictate manifestation of the two opposite insulin secretion defects by altering the expression levels of the disease mutants. ..
  2. Inagaki N, Gonoi T, Clement J, Wang C, Aguilar Bryan L, Bryan J, et al. A family of sulfonylurea receptors determines the pharmacological properties of ATP-sensitive K+ channels. Neuron. 1996;16:1011-7 pubmed
    ..The present study shows that the ATP sensitivity and pharmacological properties of K(ATP) channels are determined by a family of structurally related but functionally distinct sulfonylurea receptors...
  3. Pratt E, Tewson P, Bruederle C, Skach W, Shyng S. N-terminal transmembrane domain of SUR1 controls gating of Kir6.2 by modulating channel sensitivity to PIP2. J Gen Physiol. 2011;137:299-314 pubmed publisher
    ..2 gating by controlling channel sensitivity to PIP(2). Moreover, they uncover a novel mechanism of K(ATP) channel inactivation involving aberrant functional coupling between SUR1 and Kir6.2...
  4. Jiang K, Wang J, Zhao C, Feng M, Shen Z, Yu Z, et al. Regulation of gap junctional communication by astrocytic mitochondrial K(ATP) channels following neurotoxin administration in in vitro and in vivo models. Neurosignals. 2011;19:63-74 pubmed publisher
  5. van Bever L, Poitry S, Faure C, Norman R, Roatti A, Baertschi A. Pore loop-mutated rat KIR6.1 and KIR6.2 suppress KATP current in rat cardiomyocytes. Am J Physiol Heart Circ Physiol. 2004;287:H850-9 pubmed
    ..x trafficking to the sarcolemma. The results favor the view that KIR6.1 may associate with KIR6.2 to form heterotetrameric pores of native K(ATP) channels in cardiomyocytes. ..
  6. Proks P, Antcliff J, Lippiat J, Gloyn A, Hattersley A, Ashcroft F. Molecular basis of Kir6.2 mutations associated with neonatal diabetes or neonatal diabetes plus neurological features. Proc Natl Acad Sci U S A. 2004;101:17539-44 pubmed
    ..Heterozygous mutations in the human Kir6.2 gene (KCNJ11), the pore-forming subunit of the ATP-sensitive (K(ATP)) channel, cause permanent neonatal diabetes mellitus (PNDM)..
  7. Lin Y, Bushman J, Yan F, Haidar S, Macmullen C, Ganguly A, et al. Destabilization of ATP-sensitive potassium channel activity by novel KCNJ11 mutations identified in congenital hyperinsulinism. J Biol Chem. 2008;283:9146-56 pubmed publisher
    ..Our studies suggest the importance of Kir6.2 subunit-subunit interactions in K(ATP) channel gating and function and reveal a novel gating defect underlying CHI...
  8. Zoga V, Kawano T, Liang M, Bienengraeber M, Weihrauch D, McCallum B, et al. KATP channel subunits in rat dorsal root ganglia: alterations by painful axotomy. Mol Pain. 2010;6:6 pubmed publisher
  9. Lin C, Lin Y, Yan F, Casey J, Kochhar M, Pratt E, et al. Kir6.2 mutations associated with neonatal diabetes reduce expression of ATP-sensitive K+ channels: implications in disease mechanism and sulfonylurea therapy. Diabetes. 2006;55:1738-46 pubmed
    ..Interestingly, sulfonylureas significantly increase surface expression of certain PNDM mutants, suggesting that the efficacy of sulfonylurea therapy may be compromised by the effect of these drugs on channel expression. ..

More Information


  1. Dhar Chowdhury P, Harrell M, Han S, Jankowska D, Parachuru L, Morrissey A, et al. The glycolytic enzymes, glyceraldehyde-3-phosphate dehydrogenase, triose-phosphate isomerase, and pyruvate kinase are components of the K(ATP) channel macromolecular complex and regulate its function. J Biol Chem. 2005;280:38464-70 pubmed
    ..Our data are consistent with the concept that the activity of these enzymes (possibly by ATP formation in the immediate intracellular microenvironment of this macromolecular K(ATP) channel complex) causes channel closure. ..
  2. Thomzig A, Laube G, Prüss H, Veh R. Pore-forming subunits of K-ATP channels, Kir6.1 and Kir6.2, display prominent differences in regional and cellular distribution in the rat brain. J Comp Neurol. 2005;484:313-30 pubmed
    ..Only in distinct nuclei or neuronal subpopulations is a moderate or even strong Kir6.1 staining detected. The biological functions of these K-ATP channels still need to be elucidated...
  3. Gloyn A, Pearson E, Antcliff J, Proks P, Bruining G, Slingerland A, et al. Activating mutations in the gene encoding the ATP-sensitive potassium-channel subunit Kir6.2 and permanent neonatal diabetes. N Engl J Med. 2004;350:1838-49 pubmed
    ..2 subunit of this channel (KCNJ11) cause neonatal diabetes. We sequenced the KCNJ11 gene in 29 patients with permanent neonatal diabetes...
  4. Zhou M, Tanaka O, Suzuki M, Sekiguchi M, Takata K, Kawahara K, et al. Localization of pore-forming subunit of the ATP-sensitive K(+)-channel, Kir6.2, in rat brain neurons and glial cells. Brain Res Mol Brain Res. 2002;101:23-32 pubmed
    ..2. Under the electron microscope, we showed in vivo for the first time that the immunoreactive products were localized in the endoplasmic reticulum and Golgi apparatus as well as the plasma membranes of neurons and glial cells. ..
  5. Yan F, Casey J, Shyng S. Sulfonylureas correct trafficking defects of disease-causing ATP-sensitive potassium channels by binding to the channel complex. J Biol Chem. 2006;281:33403-13 pubmed
    ..Our results provide insight into the mechanistic and structural basis on which sulfonylureas rescue K(ATP) channel surface expression defects caused by channel mutations. ..
  6. Singh H, Hudman D, Lawrence C, Rainbow R, Lodwick D, Norman R. Distribution of Kir6.0 and SUR2 ATP-sensitive potassium channel subunits in isolated ventricular myocytes. J Mol Cell Cardiol. 2003;35:445-59 pubmed
    ..Combinations of these subunits would not explain the reported pharmacology of the mitochondrial K(ATP) channel (Mol Pharmacol 59 (2001) 225) suggesting the possibility of further unidentified components of this channel...
  7. Moritz W, Leech C, Ferrer J, Habener J. Regulated expression of adenosine triphosphate-sensitive potassium channel subunits in pancreatic beta-cells. Endocrinology. 2001;142:129-38 pubmed
    ..2 minigene by about 2-fold. We propose that glucose- and GLP-1-dependent regulation of K(ATP) subunit genes may be important in the adaptation of beta-cells to changes in secretory demands in physiological and diseased states...
  8. Yamauchi K, Stone A, Stocker S, Kaufman M. Blockade of ATP-sensitive potassium channels prevents the attenuation of the exercise pressor reflex by tempol in rats with ligated femoral arteries. Am J Physiol Heart Circ Physiol. 2012;303:H332-40 pubmed publisher
    ..We conclude that tempol attenuated both reflexes by opening K(ATP) channels, an effect that hyperpolarized muscle afferents stimulated by static contraction or tendon stretch. ..
  9. Ploug K, Amrutkar D, Baun M, Ramachandran R, Iversen A, Lund T, et al. K(ATP) channel openers in the trigeminovascular system. Cephalalgia. 2012;32:55-65 pubmed publisher
    ..Furthermore, we examined whether K(ATP) channel openers stimulate the in vitro release of CGRP and whether they degranulate dural mast cells...
  10. Wang J, Li Z, Feng M, Ren K, Shen G, Zhao C, et al. Opening of astrocytic mitochondrial ATP-sensitive potassium channels upregulates electrical coupling between hippocampal astrocytes in rat brain slices. PLoS ONE. 2013;8:e56605 pubmed publisher
    ..In addition, this effect is mainly via up-regulation of the Connexin43-constituted gap junction coupling by an ERK-dependent mechanism in the mitochondria. ..
  11. Gyte A, Pritchard L, Jones H, Brennand J, White A. Reduced expression of the KATP channel subunit, Kir6.2, is associated with decreased expression of neuropeptide Y and agouti-related protein in the hypothalami of Zucker diabetic fatty rats. J Neuroendocrinol. 2007;19:941-51 pubmed
    ..Overall, these data suggest that chronic changes in hypothalamic Kir6.2 expression may be associated with the development of hyperinsulinaemia and hyperglycaemia in ZDF rats. ..
  12. Tokuyama Y, Fan Z, Furuta H, Makielski J, Polonsky K, Bell G, et al. Rat inwardly rectifying potassium channel Kir6.2: cloning electrophysiological characterization, and decreased expression in pancreatic islets of male Zucker diabetic fatty rats. Biochem Biophys Res Commun. 1996;220:532-8 pubmed
    ..Thus, decreased expression of Kir6.2 could contribute to the beta-cell dysfunction which characterizes diabetes mellitus in this animal model. ..
  13. Hong M, Kefaloyianni E, Bao L, Malester B, Delaroche D, Neubert T, et al. Cardiac ATP-sensitive K+ channel associates with the glycolytic enzyme complex. FASEB J. 2011;25:2456-67 pubmed publisher
    ..Overall, our data demonstrate close physical association and functional interaction of the glycolytic process (particularly the distal ATP-generating steps) with cardiac K(ATP) channels...
  14. Zhou M, Tanaka O, Sekiguchi M, He H, Yasuoka Y, Itoh H, et al. ATP-sensitive K+-channel subunits on the mitochondria and endoplasmic reticulum of rat cardiomyocytes. J Histochem Cytochem. 2005;53:1491-500 pubmed
    ..The data obtained in this study will be useful for analyzing the composition of K(ATP) channels of cardiomyocytes and help to understanding the cardioprotective role of K(ATP) channels during heart ischemia...
  15. Thomas P, Ye Y, Lightner E. Mutation of the pancreatic islet inward rectifier Kir6.2 also leads to familial persistent hyperinsulinemic hypoglycemia of infancy. Hum Mol Genet. 1996;5:1809-12 pubmed
    ..Mutation of Kir6.2, like SUR, appears to lead to the PHHI phenotype suggesting that Kir6.2 is necessary, although not sufficient, for normal regulation of insulin release. ..
  16. Gloyn A, Cummings E, Edghill E, Harries L, Scott R, Costa T, et al. Permanent neonatal diabetes due to paternal germline mosaicism for an activating mutation of the KCNJ11 Gene encoding the Kir6.2 subunit of the beta-cell potassium adenosine triphosphate channel. J Clin Endocrinol Metab. 2004;89:3932-5 pubmed
    Activating mutations in the KCNJ11 gene encoding for the Kir6.2 subunit of the beta-cell ATP-sensitive potassium channel have recently been shown to be a common cause of permanent neonatal diabetes...
  17. Wang W, Li J, Meng X, Chen Y. Effect of electronic stimulation at Neiguan (PC 6) acupoint on gene expression of adenosine triphosphate-sensitive potassium channel and protein kinases in rats with myocardial ischemia. J Tradit Chin Med. 2015;35:577-82 pubmed
    ..05). Our findings suggest that electronic needling of Neiguan (PC 6) can both reduce the gene expression of KATP and protein kinases in rats with ISO-induced MI. ..
  18. Chen F, Zhang N, Liu P, Zhang Y, Han X, Cai J. [Effects of Guanxinkang on expressions of ATP-sensitive potassium channel subunits Kir6.1, Kir6.2, SUR2A and SUR2B in ischemic myocytes of rats]. Zhong Xi Yi Jie He Xue Bao. 2010;8:458-64 pubmed
  19. Yin X, Fu Z, Zhang D, Jiang B. Alterations in the expression of ATP-sensitive potassium channel subunit mRNA after acute peripheral nerve and spinal cord injury. Eur Neurol. 2007;57:4-10 pubmed
    ..These results suggest that acute peripheral nerve and spinal cord injury provoke different regulations of K(ATP) gene expression in the peripheral and central nervous system. ..
  20. Fan X, Ding Y, Cheng H, Gram D, Sherwin R, McCrimmon R. Amplified hormonal counterregulatory responses to hypoglycemia in rats after systemic delivery of a SUR-1-selective K(+) channel opener?. Diabetes. 2008;57:3327-34 pubmed publisher
    ..2/SUR-1 isoform of the K(ATP) channel, we sought to examine the therapeutic potential of the SUR-1-selective potassium channel opener (KCO), NN414, to amplify counterregulatory response to hypoglycemia...
  21. Bancila V, Cens T, Monnier D, Chanson F, Faure C, Dunant Y, et al. Two SUR1-specific histidine residues mandatory for zinc-induced activation of the rat KATP channel. J Biol Chem. 2005;280:8793-9 pubmed
    ..In fact, we have recently shown that such a mechanism occurs in hippocampal mossy fibers, a brain region characterized, like the pancreas, by an important accumulation of zinc and a high density of SUR1-containing KATP...
  22. Lybaert P, Hoofd C, Guldner D, Vegh G, Delporte C, Meuris S, et al. Detection of K(ATP) channels subunits in human term placental explants and evaluation of their implication in human placental lactogen (hPL) and human chorionic gonadotropin (hCG) release. Placenta. 2013;34:467-73 pubmed publisher
    ..The present findings suggest that the human term syncytiotrophoblast might be endowed with KATP channels. Further studies should clarify their implication in the syncytiotrophoblast ionic homeostasis and hormone regulation. ..
  23. Shimizu S, Saito M, Kinoshita Y, Ohmasa F, Dimitriadis F, Shomori K, et al. Nicorandil ameliorates ischaemia-reperfusion injury in the rat kidney. Br J Pharmacol. 2011;163:272-82 pubmed publisher
    ..Nicorandil could represent a powerful additional component in the treatment of patients undergoing partial nephrectomy or renal transplantation. ..
  24. Chen Z, Cheng Y, Chen L, Cheng K, Li Y, Cheng J. Increase of ATP-sensitive potassium (K(ATP)) channels in the heart of type-1 diabetic rats. Cardiovasc Diabetol. 2012;11:8 pubmed publisher
    ..It is highly responsive to metabolic fluctuations and can have effects on cardiac contractility. The present study attempted to clarify the changes of cardiac K(ATP) channels in diabetic disorders...
  25. Cooper P, McClenaghan C, Chen X, Stary Weinzinger A, Nichols C. 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;292:17387-17398 pubmed publisher
    ..This raises the possibility that, at least for some CS mutations, sulfonylurea therapy may not prove to be successful and highlights the need for detailed pharmacogenomic analyses of CS mutations. ..
  26. Morrissey A, Rosner E, Lanning J, Parachuru L, Dhar Chowdhury P, Han S, et al. Immunolocalization of KATP channel subunits in mouse and rat cardiac myocytes and the coronary vasculature. BMC Physiol. 2005;5:1 pubmed
    ..2 and SUR2A subunits, but the distribution of these (and other KATP channel subunits) is poorly defined. We examined the localization of each of the KATP channel subunits in the mouse and rat heart...
  27. Wan T, Ge Z, Tampo A, Mio Y, Bienengraeber M, Tracey W, et al. The A3 adenosine receptor agonist CP-532,903 [N6-(2,5-dichlorobenzyl)-3'-aminoadenosine-5'-N-methylcarboxamide] protects against myocardial ischemia/reperfusion injury via the sarcolemmal ATP-sensitive potassium channel. J Pharmacol Exp Ther. 2008;324:234-43 pubmed
    ..We conclude that CP-532,903 is a highly selective agonist of the mouse A(3)AR that protects against ischemia/reperfusion injury by activating sarcolemmal K(ATP) channels. ..
  28. Lodwick D, Rainbow R, Rubaiy H, Al Johi M, Vuister G, Norman R. Sulfonylurea receptors regulate the channel pore in ATP-sensitive potassium channels via an intersubunit salt bridge. Biochem J. 2014;464:343-54 pubmed publisher
    ..Equivalent interactions were identified in Kir6.1- and Kir6.2-containing channels suggesting a conserved mechanism of allosteric regulation. ..
  29. Martin G, Yoshioka C, Rex E, Fay J, Xie Q, Whorton M, et al. Cryo-EM structure of the ATP-sensitive potassium channel illuminates mechanisms of assembly and gating. elife. 2017;6: pubmed publisher
  30. Hong M, Bao L, Kefaloyianni E, Agullo Pascual E, Chkourko H, Foster M, et al. Heterogeneity of ATP-sensitive K+ channels in cardiac myocytes: enrichment at the intercalated disk. J Biol Chem. 2012;287:41258-67 pubmed publisher
    ..Our data demonstrate heterogeneity of K(ATP) channel distribution within a cardiac myocyte. The higher K(ATP) channel density at the intercalated disk implies a possible role at the intercellular junctions during cardiac ischemia. ..
  31. Lim A, Park S, Sohn J, Jeon J, Park J, Song D, et al. Glucose deprivation regulates KATP channel trafficking via AMP-activated protein kinase in pancreatic beta-cells. Diabetes. 2009;58:2813-9 pubmed publisher
    ..K(ATP) channel trafficking is regulated by energy status via AMPK, and this mechanism may play a key role in inhibiting insulin secretion under low energy status. ..
  32. Jovanović S, Du Q, Sukhodub A, Jovanović A. M-LDH physically associated with sarcolemmal K ATP channels mediates cytoprotection in heart embryonic H9C2 cells. Int J Biochem Cell Biol. 2009;41:2295-301 pubmed publisher
    ..2/SUR2A regulates the activity of sarcolemmal K(ATP) channels as well as an intracellular ATP production during metabolic stress, both of which are important for cell survival. ..
  33. Kline C, Kurata H, Hund T, Cunha S, Koval O, Wright P, et al. Dual role of K ATP channel C-terminal motif in membrane targeting and metabolic regulation. Proc Natl Acad Sci U S A. 2009;106:16669-74 pubmed publisher
    ..Thus, the K(ATP) channel ABM serves as a previously unrecognized bifunctional touch-point for grading K(ATP) channel gating and membrane targeting and may play a fundamental role in controlling excitable cell metabolic regulation. ..
  34. Wang S, Hu L, Yang Y, Ding J, Hu G. Studies of ATP-sensitive potassium channels on 6-hydroxydopamine and haloperidol rat models of Parkinson's disease: implications for treating Parkinson's disease?. Neuropharmacology. 2005;48:984-92 pubmed
    ..Our results suggest that KATP channels might be involved in the pathogenesis of Parkinson's disease (PD) induced in an animal model and conceptually support the idea that KATP channels may be new therapeutic targets for PD. ..
  35. Girard C, Wunderlich F, Shimomura K, Collins S, Kaizik S, Proks P, et al. Expression of an activating mutation in the gene encoding the KATP channel subunit Kir6.2 in mouse pancreatic beta cells recapitulates neonatal diabetes. J Clin Invest. 2009;119:80-90 pubmed publisher
    ..2, SUR1, and insulin mRNA. All these changes are expected to contribute to the diabetes of beta-V59M mice. Their cause requires further investigation. ..
  36. Gloyn A, Siddiqui J, Ellard S. Mutations in the genes encoding the pancreatic beta-cell KATP channel subunits Kir6.2 (KCNJ11) and SUR1 (ABCC8) in diabetes mellitus and hyperinsulinism. Hum Mutat. 2006;27:220-31 pubmed
    ..It has been known for some time that loss of function mutations in KCNJ11, which encodes for Kir6...
  37. Riedel M, Steckley D, Light P. Current status of the E23K Kir6.2 polymorphism: implications for type-2 diabetes. Hum Genet. 2005;116:133-45 pubmed
  38. Tsounapi P, Saito M, Dimitriadis F, Kitatani K, Kinoshita Y, Shomori K, et al. The role of K ATP channels on ischemia-reperfusion injury in the rat testis. Life Sci. 2012;90:649-56 pubmed publisher
    ..This is the first study to give evidence for the advantageous effect of cromakalim in the germ cell-specific apoptosis induced by testicular IR. ..
  39. Gaber E, Jayaprakash P, Qureshi M, Parekh K, Oz M, Adrian T, et al. Effects of a sucrose-enriched diet on the pattern of gene expression, contraction and Ca(2+) transport in Goto-Kakizaki type 2 diabetic rat heart. Exp Physiol. 2014;99:881-93 pubmed publisher
    ..Gja4), cell membrane transport (Atp1b1), calcium channels (Cacna1c, Cacna1g and Cacnb1) and potassium channels (Kcnj11) were upregulated and genes encoding potassium channels (Kcnb1) were downregulated in GK compared with control ..
  40. Xia H, Zhang D, Yang S, Wang Y, Xu L, Wu J, et al. Role of ATP-sensitive potassium channels in modulating nociception in rat model of bone cancer pain. Brain Res. 2014;1554:29-35 pubmed publisher
    ..Our findings suggest that the KATP channel expression level in the spinal cord is reduced in bone cancer pain. Activation of KATP channels at the spinal level reduces pain hypersensitivity associated with bone cancer pain. ..
  41. Pratt E, Zhou Q, Gay J, Shyng S. Engineered interaction between SUR1 and Kir6.2 that enhances ATP sensitivity in KATP channels. J Gen Physiol. 2012;140:175-87 pubmed publisher
    ..Our results reveal that ATP sensitivity in K(ATP) channels is a dynamic parameter dictated by interactions between SUR1 and Kir6.2...
  42. Huang C, Lai M, Cheng J, Tsai J, Huang C, Wu S. Pregabalin attenuates excitotoxicity in diabetes. PLoS ONE. 2013;8:e65154 pubmed publisher
    ..These findings might help develop a clinical algorithm for treating patients with epilepsy and comorbid metabolic disorders. ..
  43. Zhang X, Zhang X, Xiong Y, Xu C, Liu X, Lin J, et al. Sarcolemmal ATP-sensitive potassium channel protects cardiac myocytes against lipopolysaccharide-induced apoptosis. Int J Mol Med. 2016;38:758-66 pubmed publisher
    ..Elucidating the regulatory mechanisms of the sarcKATP channel in apoptosis may facilitate the development of novel therapeutic targets and strategies for the management of sepsis and cardiac dysfunction. ..
  44. Dong K, Tang L, MacGregor G, Leng Q, Hebert S. Novel nucleotide-binding sites in ATP-sensitive potassium channels formed at gating interfaces. EMBO J. 2005;24:1318-29 pubmed
    ..The short N- and C-terminal segments comprising the novel intermolecular NBS are next to helices that likely move with channel opening/closing, suggesting a lock-and-key model for ligand gating. ..
  45. Faletra F, Athanasakis E, Morgan A, Biarnés X, Fornasier F, Parini R, et al. Congenital hyperinsulinism: clinical and molecular analysis of a large Italian cohort. Gene. 2013;521:160-5 pubmed publisher
    ..two major subgroups: "channelopathies" due to defects in ATP-sensitive potassium channel, encoded by ABCC8 and KCNJ11 genes, and "metabolopathies" caused by mutation of several genes (GLUD1, GCK, HADH, SLC16A1, HNF4A and HNF1A) and ..
  46. Storey N, Stratton R, Rainbow R, Standen N, Lodwick D. Kir6.2 limits Ca(2+) overload and mitochondrial oscillations of ventricular myocytes in response to metabolic stress. Am J Physiol Heart Circ Physiol. 2013;305:H1508-18 pubmed publisher
    ..KATP channel density is important for adaption to metabolic stress. ..
  47. Florez J, Jablonski K, Kahn S, Franks P, Dabelea D, Hamman R, et al. Type 2 diabetes-associated missense polymorphisms KCNJ11 E23K and ABCC8 A1369S influence progression to diabetes and response to interventions in the Diabetes Prevention Program. Diabetes. 2007;56:531-6 pubmed
    The common polymorphisms KCNJ11 E23K and ABCC8 A1369S have been consistently associated with type 2 diabetes...
  48. Craig T, Shimomura K, Holl R, Flanagan S, Ellard S, Ashcroft F. An in-frame deletion in Kir6.2 (KCNJ11) causing neonatal diabetes reveals a site of interaction between Kir6.2 and SUR1. J Clin Endocrinol Metab. 2009;94:2551-7 pubmed publisher
    Activating mutations in genes encoding the Kir6.2 (KCNJ11) and SUR1 (ABCC8) subunits of the pancreatic ATP-sensitive K(+) channel are a common cause of permanent neonatal diabetes (PNDM). All Kir6...
  49. Li L, Rojas A, Wu J, Jiang C. Disruption of glucose sensing and insulin secretion by ribozyme Kir6.2-gene targeting in insulin-secreting cells. Endocrinology. 2004;145:4408-14 pubmed
    ..2-gene targeting is an effective approach for selective inhibition of functional expression of KATP channels. ..
  50. Liang T, Xie L, Chao C, Kang Y, Lin X, Qin T, et al. Phosphatidylinositol 4,5-biphosphate (PIP2) modulates interaction of syntaxin-1A with sulfonylurea receptor 1 to regulate pancreatic ?-cell ATP-sensitive potassium channels. J Biol Chem. 2014;289:6028-40 pubmed publisher
    ..These results taken together indicate that PIP2 affects islet ?-cell KATP channels not only by its actions on Kir6.2 but also by sequestering Syn-1A to modulate Syn-1A availability and its interactions with SUR1 on PM. ..
  51. Rainbow R, James M, Hudman D, Al Johi M, Singh H, Watson P, et al. Proximal C-terminal domain of sulphonylurea receptor 2A interacts with pore-forming Kir6 subunits in KATP channels. Biochem J. 2004;379:173-81 pubmed
    ..These results identify a site in the C-terminal domain of rSUR2A, between residues 1294 and 1358, whose direct interaction with full-length Kir6.2 is crucial for the assembly of functional KATP channels...
  52. Cao K, Tang G, Hu D, Wang R. Molecular basis of ATP-sensitive K+ channels in rat vascular smooth muscles. Biochem Biophys Res Commun. 2002;296:463-9 pubmed
    ..Our results for the first time reported the expression of four K(ATP) subunits in same vascular tissues, unmasking the diversity of native K(ATP) channels in vascular SMCs. ..
  53. Ye D, Zhou W, Lu T, Jagadeesh S, Falck J, Lee H. Mechanism of rat mesenteric arterial KATP channel activation by 14,15-epoxyeicosatrienoic acid. Am J Physiol Heart Circ Physiol. 2006;290:H1326-36 pubmed
    ..These results suggest that 14,15-EET activates vascular K(ATP) channels through ADP-ribosylation of G(s)alpha. ..
  54. Morrissey A, Parachuru L, Leung M, Lopez G, Nakamura T, Tong X, et al. Expression of ATP-sensitive K+ channel subunits during perinatal maturation in the mouse heart. Pediatr Res. 2005;58:185-92 pubmed
    ..Our data suggest that the K(ATP) channel composition may change during maturation, which has important implications for K(ATP) channel function in the developing heart...
  55. Isidoro Tavares N, Philip Couderc P, Baertschi A, Lerch R, Montessuit C. Angiotensin II and tumour necrosis factor alpha as mediators of ATP-dependent potassium channel remodelling in post-infarction heart failure. Cardiovasc Res. 2009;83:726-36 pubmed publisher
    ..Here, we test their role in the remodelling of ATP-dependent potassium channel (K(ATP)) in heart failure, conferring increased metabolic and diazoxide sensitivity...
  56. Wu S, Wu A, Sung R. Identification of two types of ATP-sensitive K+ channels in rat ventricular myocytes. Life Sci. 2007;80:378-87 pubmed
    ..The present results suggest that these two types of K(ATP) channels may functionally be related to the activity of heart cells. ..
  57. Albaqumi M, Alhabib F, Shamseldin H, Mohammed F, Alkuraya F. A syndrome of congenital hyperinsulinism and rhabdomyolysis is caused by KCNJ11 mutation. J Med Genet. 2014;51:271-4 pubmed publisher
    ..To describe significant skeletal muscle manifestations in a family with a novel mutation in KCNJ11 (encoding the Kir6.2 component of K(ATP))...
  58. Zhou S, Lu Y, Song I, Owyang C. Inhibition of gastric motility by hyperglycemia is mediated by nodose ganglia KATP channels. Am J Physiol Gastrointest Liver Physiol. 2011;300:G394-400 pubmed publisher
    ..These studies provide in vivo evidence that hyperglycemia induces gastric relaxation via the vagal afferent pathway. This action is mediated through inactivation of nodose ganglia K(ATP) channels. ..
  59. Zhou Q, Pratt E, Shyng S. Engineered Kir6.2 mutations that correct the trafficking defect of K(ATP) channels caused by specific SUR1 mutations. Channels (Austin). 2013;7:313-7 pubmed
    ..2. Our study demonstrates for the first time that engineered mutations in Kir6.2 can correct the biogenesis defect caused by specific mutations in the SUR1 subunit. ..
  60. Zhou M, He H, Tanaka O, Sekiguchi M, Kawahara K, Abe H. Different localization of ATP sensitive K+ channel subunits in rat testis. Anat Rec (Hoboken). 2011;294:729-37 pubmed publisher
    ..Different localizations of the K(ATP) channel subunits in the cell membrane and membranous organelles of spermatogenic cells and Sertoli cells indicated the complex and multiple functions of K(ATP) channels in rat testis...
  61. Moran O, Grottesi A, Chadburn A, Tammaro P. Parametrisation of the free energy of ATP binding to wild-type and mutant Kir6.2 potassium channels. Biophys Chem. 2013;171:76-83 pubmed publisher
    ..2 channel. A set of LIE parameters was defined that may enable prediction of the effects of additional Kir6.2 mutations within the ATP binding site on the affinity for ATP. ..
  62. Gloyn A, Diatloff Zito C, Edghill E, Bellanne Chantelot C, Nivot S, Coutant R, et al. KCNJ11 activating mutations are associated with developmental delay, epilepsy and neonatal diabetes syndrome and other neurological features. Eur J Hum Genet. 2006;14:824-30 pubmed
    Heterozygous activating mutations in the gene encoding for the ATP-sensitive potassium channel subunit Kir6.2 (KCNJ11) have recently been shown to be a common cause of permanent neonatal diabetes. Kir6...
  63. Hiriart M, Aguilar Bryan L. Channel regulation of glucose sensing in the pancreatic beta-cell. Am J Physiol Endocrinol Metab. 2008;295:E1298-306 pubmed publisher
  64. Chen B, Wu S. Functional role of the activity of ATP-sensitive potassium channels in electrical behavior of hippocampal neurons: experimental and theoretical studies. J Theor Biol. 2011;272:16-25 pubmed publisher
    ..Taken together, it is anticipated that the increased activity of K(ATP) channels caused by increasing N(O) or ?(bas) contributes to or is responsible for burst firing of APs in hippocampal neurons if similar results occur in vivo. ..
  65. Bao L, Hadjiolova K, Coetzee W, Rindler M. Endosomal KATP channels as a reservoir after myocardial ischemia: a role for SUR2 subunits. Am J Physiol Heart Circ Physiol. 2011;300:H262-70 pubmed publisher
    ..In cardiac myocytes, this pool can potentially play a cardioprotective role by serving as a reservoir for modulating surface K(ATP) channel density under stress conditions, such as myocardial ischemia...
  66. Sun W, Hu K. Role for SUR2A in coupling cardiac K(ATP) channels to caveolin-3. Cell Physiol Biochem. 2010;25:409-18 pubmed publisher
    ..We concluded that SUR2A is important for coupling cardiac K(ATP) channels to caveolin-3, possibly through the caveolin-3 scaffolding domain...
  67. Grabauskas G, Song I, Zhou S, Owyang C. Electrophysiological identification of glucose-sensing neurons in rat nodose ganglia. J Physiol. 2010;588:617-32 pubmed publisher
    ..The results also show that PIP(2) can determine the excitability of glucose-excited neurons...
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    ..2 correlate with distinct positions of TMD0. They further demonstrate that the second intracellular loop of SUR, which contains the two residues studied here, is a key structural element of the TMD0-Kir6.2 interface...
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    ..The majority of mutations have been identified in the ABCC8 and KCNJ11 genes encoding subunits of the KATP channel responsible for two distinct histological forms...
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    ..We determined the expression levels of genes Hsd11b1, Nr3c1, Abcc8, Kcnj11, Mc4r, Npy, Lepr, Bdnf, and Drd2 that are involved in regulation of energy metabolism, in the hippocampus of age-..
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    ..05). These results indicated that K(ATP) channels in brain particularly in DG are likely related to enhanced seizure susceptibility and dynamic controls of seizure propagation of chronic epilepsy induced by PTX in rats. ..