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ATP-sensitive potassium channelopathies: focus on insulin secretion
Frances M. Ashcroft
Frances M. Ashcroft
Published August 1, 2005
Citation Information: J Clin Invest. 2005;115(8):2047-2058. https://doi.org/10.1172/JCI25495.
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ATP-sensitive potassium channelopathies: focus on insulin secretion

Abstract

ATP-sensitive potassium (KATP) channels, so named because they are inhibited by intracellular ATP, play key physiological roles in many tissues. In pancreatic β cells, these channels regulate glucose-dependent insulin secretion and serve as the target for sulfonylurea drugs used to treat type 2 diabetes. This review focuses on insulin secretory disorders, such as congenital hyperinsulinemia and neonatal diabetes, that result from mutations in KATP channel genes. It also considers the extent to which defective regulation of KATP channel activity contributes to the etiology of type 2 diabetes.

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Frances M. Ashcroft

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Incretin Therapies Do Not Expand β-Cell Mass or Alter Pancreatic Histology in Young Male Mice
Cox AR, Lam CJ, Rankin MM, Rios JS, Chavez J, Bonnyman CW, King KB, Wells RA, Anthony D, Tu JX, Kim JJ, Li C, Kushner JA 		Endocrinology 2017
Glucose elicits cephalic-phase insulin release in mice by activating KATP channels in taste cells
Glendinning JI, Frim YG, Hochman A, Lubitz GS, Basile AJ, Sclafani A 		American Journal of Physiology - Regulatory, Integrative and Comparative Physiology 2017
Cryo-EM structure of the ATP-sensitive potassium channel illuminates mechanisms of assembly and gating
Martin GM, Yoshioka C, Rex EA, Fay JF, Xie Q, Whorton MR, Chen JZ, Shyng SL 		eLife 2017
From ions to insulin
Kanelis V 		eLife 2017
High glucose stimulates cell proliferation and Collagen IV production in rat mesangial cells through inhibiting AMPK-K(ATP) signaling.
Zhang B, Shi YQ, Zou JJ, Chen XF, Tang W, Ye F, Liu ZM 		International Urology and Nephrology 2017
NOX4-dependent fatty acid oxidation promotes NLRP3 inflammasome activation in macrophages
JS Moon, K Nakahira, KP Chung, GM DeNicola, MJ Koo, MA Pabón, KT Rooney, JH Yoon, SW Ryter, H Stout-Delgado, AM Choi 		Nature Medicine 2016
Hyperinsulinemic Hypoglycemia – The Molecular Mechanisms
A Nessa, SA Rahman, K Hussain 		Frontiers in Endocrinology 2016
Short-Term High-Fat Diet (HFD) Induced Anxiety-Like Behaviors and Cognitive Impairment Are Improved with Treatment by Glyburide
SJ Gainey, KA Kwakwa, JK Bray, MM Pillote, VL Tir, AE Towers, GG Freund 		Frontiers in behavioral neuroscience 2016
The Pathogenic Role of NLRP3 Inflammasome Activation in Inflammatory Bowel Diseases of Both Mice and Humans
L Liu, Y Dong, M Ye, S Jin, J Yang, ME Joosse, Y Sun, J Zhang, M Lazarev, SR Brant, B Safar, M Marohn, E Mezey, X Li 		Journal of Crohn's & colitis 2016
A Novel Diphenylthiosemicarbazide Is a Potential Insulin Secretagogue for Anti-Diabetic Agent
Sugawara K, Honda K, Reien Y, Yokoi N, Seki C, Takahashi H, Minami K, Mori I, Matsumoto A, Nakaya H, Seino S 		PloS one 2016
Pharmacological Correction of Trafficking Defects in ATP-sensitive Potassium Channels Caused by Sulfonylurea Receptor 1 Mutations
Martin GM, Rex EA, Devaraneni P, Denton JS, Boodhansingh KE, DeLeon DD, Stanley CA, Shyng SL 		The Journal of biological chemistry 2016
A high-sensitivity HPLC assay for measuring intracellular Na+ and K+ and its application to Plasmodium falciparum infected erythrocytes
Winterberg M, Kirk K 		Scientific Reports 2016
CD200 Inhibits Inflammatory Response by Promoting KATP Channel Opening in Microglia Cells in Parkinson’s Disease
Ren Y, Ye M, Chen S, Ding J 		Medical science monitor : international medical journal of experimental and clinical research 2016
Potassium and Obesity/Metabolic Syndrome: A Systematic Review and Meta-Analysis of the Epidemiological Evidence
Cai X, Li X, Fan W, Yu W, Wang S, Li Z, Scott EM, Li X 		Nutrients 2016
Isosteviol Sensitizes sarcKATP Channels towards Pinacidil and Potentiates Mitochondrial Uncoupling of Diazoxide in Guinea Pig Ventricular Myocytes
Fan Z, Wen T, Chen Y, Huang L, Lin W, Yin C, Tan W 		Oxidative medicine and cellular longevity 2016
Neuronal and Cardiovascular Potassium Channels as Therapeutic Drug Targets: Promise and Pitfalls
ES Humphries, C Dart 		Journal of biomolecular screening 2015
Beta Cell Function and the Nutritional State: Dietary Factors that Influence Insulin Secretion
WT Moore, SM Bowser, DW Fausnacht, LL Staley, KS Suh, D Liu 		Current Diabetes Reports 2015
A Role for Astrocytes in Sensing the Brain Microenvironment and Neuro-Metabolic Integration
AG Teschemacher, AV Gourine, S Kasparov 		Neurochemical Research 2015
Volatile anesthetics suppress glucose-stimulated insulin secretion in MIN6 cells by inhibiting glucose-induced activation of hypoxia-inducible factor 1
K Suzuki, Y Sato, S Kai, K Nishi, T Adachi, Y Matsuo, K Hirota 		PeerJ 2015
A small-molecule inhibitor of the NLRP3 inflammasome for the treatment of inflammatory diseases
RC Coll, AA Robertson, JJ Chae, SC Higgins, R Muñoz-Planillo, MC Inserra, I Vetter, LS Dungan, BG Monks, A Stutz, DE Croker, MS Butler, M Haneklaus, CE Sutton, G Núñez, E Latz, DL Kastner, KH Mills, SL Masters, K Schroder, MA Cooper, LA O'Neill 		Nature Medicine 2015
ABCC8 R1420H Loss-of-Function Variant in a Southwest American Indian Community: Association With Increased Birth Weight and Doubled Risk of Type 2 Diabetes
LJ Baier, YL Muller, MS Remedi, M Traurig, P Piaggi, G Wiessner, K Huang, A Stacy, S Kobes, J Krakoff, PH Bennett, RG Nelson, WC Knowler, RL Hanson, CG Nichols, C Bogardus 		Diabetes 2015
Intractable hyperkalemia due to nicorandil induced potassium channel syndrome
Chowdhry V, Mohanty BB 		Annals of Cardiac Anaesthesia 2015
Concerted Trafficking Regulation of Kv2.1 and KATP Channels by Leptin in Pancreatic β-Cells
Wu Y, Shyng SL, Chen PC 		The Journal of biological chemistry 2015
Therapeutics Targeting the Inflammasome After Central Nervous System Injury
de Rivero Vaccari JP, Dietrich WD, Keane RW 		Translational research : the journal of laboratory and clinical medicine 2015
Age at the time of sulfonylurea initiation influences treatment outcomes in KCNJ11-related neonatal diabetes
Thurber BW, Carmody D, Tadie EC, Pastore AN, Dickens JT, Wroblewski KE, Naylor RN, Philipson LH, Greeley SA 		Diabetologia 2015
Evaluation of Antidiabetic Effects of the Traditional Medicinal Plant Gynostemma pentaphyllum and the Possible Mechanisms of Insulin Release
Lokman EF, Gu HF, Wan Mohamud WN, Östenson CG 		Evidence-based complementary and alternative medicine : eCAM 2015
Evidence for a KATP Channel in Rough Endoplasmic Reticulum (rerKATP Channel) of Rat Hepatocytes
Salari S, Ghasemi M, Fahanik-Babaei J, Saghiri R, Sauve R, Eliassi A 		PloS one 2015
Structurally Distinct Ligands Rescue Biogenesis Defects of the KATP Channel Complex via a Converging Mechanism
Devaraneni PK, Martin GM, Olson EM, Zhou Q, Shyng SL 		The Journal of biological chemistry 2015
Targeting the NLRP3 inflammasome in chronic inflammatory diseases: current perspectives
Ozaki E, Campbell M, Doyle SL 		Journal of inflammation research 2015
The inwardly rectifying K+ channel KIR7.1 controls uterine excitability throughout pregnancy
C McCloskey, C Rada, E Bailey, S McCavera, HA van Berg, J Atia, DA Rand, A Shmygol, YW Chan, S Quenby, JJ Brosens, M Vatish, J Zhang, JS Denton, MJ Taggart, C Kettleborough, D Tickle, J Jerman, P Wright, T Dale, S Kanumilli, DJ Trezise, S Thornton, P Brown, R Catalano, N Lin, SK England, AM Blanks 		EMBO Molecular Medicine 2014
Congenital hyperinsulinism: clinical and molecular characterisation of compound heterozygous ABCC8 mutation responsive to Diazoxide therapy
V Arya, Q Aziz, A Nessa, A Tinker, K Hussain 		International Journal of Pediatric Endocrinology 2014
Channelopathies
JB Kim 		Korean Journal of Pediatrics 2014
Sulfonylureas: A New Look at Old Therapy
PM Thulé, G Umpierrez 		Current Diabetes Reports 2014
A Digital Atlas of Ion Channel Expression Patterns in the Two-Week-Old Rat Brain
Shcherbatyy V, Carson J, Yaylaoglu M, Jäckle K, Grabbe F, Brockmeyer M, Yavuz H, Eichele G 		Neuroinformatics 2014
Jumping on the Train of Personalized Medicine: A Primer for Non- Geneticist Clinicians: Part 3. Clinical Applications in the Personalized Medicine Area
Li A, Meyre D 		Current Psychiatry Reviews 2014
Carbamazepine inhibits ATP-sensitive potassium channel activity by disrupting channel response to MgADP
Zhou Q, Chen PC, Devaraneni PK, Martin GM, Olson EM, Shyng SL 		Channels 2014
Association between E23K variant in KCNJ11 gene and new-onset diabetes after liver transplantation.
Parvizi Z, Azarpira N, Kohan L, Darai M, Kazemi K, Parvizi MM 		Molecular Biology Reports 2014
Leptin promotes K(ATP) channel trafficking by AMPK signaling in pancreatic β-cells
SH Park, SY Ryu, WJ Yu, YE Han, YS Ji, K Oh, JW Sohn, A Lim, JP Jeon, H Lee, KH Lee, SH Lee, PO Berggren, JH Jeon, WK Ho 		Proceedings of the National Academy of Sciences 2013
Pharmacological rescue of trafficking-impaired ATP-sensitive potassium channels
GM Martin, PC Chen, P Devaraneni, SL Shyng 		Frontiers in physiology 2013
Leptin Regulates KATP Channel Trafficking in Pancreatic  -Cells by a Signaling Mechanism Involving AMP-activated Protein Kinase (AMPK) and cAMP-dependent Protein Kinase (PKA)
PC Chen, YN Kryukova, SL Shyng 		The Journal of biological chemistry 2013
Carbamazepine as a novel small molecule corrector of trafficking-impaired ATP-sensitive potassium channels identified in congenital hyperinsulinism
PC Chen, EM Olson, Q Zhou, Y Kryukova, HM Sampson, DY Thomas, SL Shyng 		The Journal of biological chemistry 2013
Pathogenesis of the metabolic syndrome: insights from monogenic disorders
R Murphy, RW Carroll, JD Krebs 		Mediators of Inflammation 2013
Effects of Sulfonylureas on Tumor Growth: A Review of the Literature
G Pasello, L Urso, P Conte, A Favaretto 		The oncologist 2013
TLR-independent and P2X7-dependent signaling mediate Alu RNA-induced NLRP3 inflammasome activation in geographic atrophy
N Kerur, Y Hirano, V Tarallo, BJ Fowler, A Bastos-Carvalho, T Yasuma, R Yasuma, Y Kim, DR Hinton, CJ Kirschning, BD Gelfand, J Ambati 		Investigative ophthalmology & visual science 2013
Control of voltage-gated potassium channel Kv2.2 expression by pyruvate-isocitrate cycling regulates glucose-stimulated insulin secretion
MV Jensen, JM Haldeman, H Zhang, D Lu, MO Huising, WW Vale, HE Hohmeier, P Rosenberg, CB Newgard 		The Journal of biological chemistry 2013
Green tea and type 2 diabetes
Park JH, Bae JH, Im SS, Song DK 		Integrative Medicine Research 2013
Engineered Kir6.2 mutations that correct the trafficking defect of KATP channels caused by specific SUR1 mutations
Zhou Q, Pratt EB, Shyng SL 		Channels 2013
Glucose-stimulated Single Pancreatic Islets Sustain Increased Cytosolic ATP Levels during Initial Ca2+ Influx and Subsequent Ca2+ Oscillations
Tanaka T, Nagashima K, Inagaki N, Kioka H, Takashima S, Fukuoka H, Noji H, Kakizuka A, Imamura H 		The Journal of biological chemistry 2013
Neonatal Diabetes Caused by Activating Mutations in the Sulphonylurea Receptor
Proks P 		Diabetes & Metabolism Journal 2013
Control of beta cell function and proliferation in mice stimulated by small-molecule glucokinase activator under various conditions
A Nakamura, Y Togashi, K Orime, K Sato, J Shirakawa, M Ohsugi, N Kubota, T Kadowaki, Y Terauchi 		Diabetologia 2012
Congenital Hyperinsulinism Caused by Hexokinase I Expression or Glucokinase-Activating Mutation in a Subset of  -Cells
JC Henquin, C Sempoux, J Marchandise, S Godecharles, Y Guiot, M Nenquin, J Rahier 		Diabetes 2012
AMP-activated protein kinase: an emerging drug target to regulate imbalances in lipid and carbohydrate metabolism to treat cardio-metabolic diseases
RA Srivastava, SL Pinkosky, S Filippov, JC Hanselman, CT Cramer, RS Newton 		Journal of lipid research 2012
MED25 Is a Mediator Component of HNF4α-Driven Transcription Leading to Insulin Secretion in Pancreatic Beta-Cells
EH Han, GB Rha, YI Chi 		PloS one 2012
Detection of KCNJ11 Gene Mutations in a Family with Neonatal Diabetes Mellitus: Implications for Therapeutic Management of Family Members with Long-Standing Disease
F Abbasi, S Saba, A Ebrahim-Habibi, FA Sayahpour, P Amiri, B Larijani, MM Amoli 		Molecular diagnosis & therapy 2012
Engineered interaction between SUR1 and Kir6.2 that enhances ATP sensitivity in KATP channels
EB Pratt, Q Zhou, JW Gay, SL Shyng 		The Journal of General Physiology 2012
Rapid Insulinotropic Action of Low Doses of Bisphenol-A on Mouse and Human Islets of Langerhans: Role of Estrogen Receptor β
S Soriano, P Alonso-Magdalena, M García-Arévalo, A Novials, SJ Muhammed, A Salehi, JA Gustafsson, I Quesada, A Nadal 		PloS one 2012
Nateglinide is Effective for Diabetes Mellitus with Reactive Hypoglycemia in a Child with a Compound Heterozygous ABCC8 Mutation
A Saito-Hakoda, T Yorifuji, J Kanno, S Kure, I Fujiwara 		Clinical Pediatric Endocrinology 2012
Heterogeneity in phenotype of usher-congenital hyperinsulinism syndrome: hearing loss, retinitis pigmentosa, and hyperinsulinemic hypoglycemia ranging from severe to mild with conversion to diabetes.
Al Mutair AN, Brusgaard K, Bin-Abbas B, Hussain K, Felimban N, Al Shaikh A, Christesen HT 		Diabetes care 2012
Compound heterozygous mutations in the SUR1 (ABCC 8) subunit of pancreatic KATP channels cause neonatal diabetes by perturbing the coupling between Kir6.2 and SUR1 subunits
Lin YW, Akrouh A, Hsu Y, Hughes N, Nichols CG, De León DD 		Channels 2012
Hyaluronan Export through Plasma Membranes Depends on Concurrent K+ Efflux by Kir Channels
Hagenfeld D, Borkenhagen B, Schulz T, Schillers H, Schumacher U, Prehm P 		PloS one 2012
Role of Derlin-1 Protein in Proteostasis Regulation of ATP-sensitive Potassium Channels
Wang F, Olson EM, Shyng SL 		The Journal of biological chemistry 2012
Insulin dynamics in obesity and diabetes
Susumu Seino, Tadao Shibasaki, Kohtaro Minami 		Journal of Clinical Investigation 2011
Elevated Hedgehog/Gli signaling causes β-cell dedifferentiation in mice
L Landsman, A Parent, M Hebrok 		Proceedings of the National Academy of Sciences 2011
Comprehensive Physiology
R Terjung 		Comprehensive Physiology 2011
Characterization of the ATP-sensitive K+ channel in the rat carotid body glomus cells
D Kim, I Kim, JR Papreck, DF Donnelly, JL Carroll 		Respiratory Physiology & Neurobiology 2011
Advances in Cardiac ATP-Sensitive K + Channelopathies From Molecules to Populations
A Terzic, AE Alekseev, S Yamada, S Reyes, TM Olson 		Circulation. Arrhythmia and electrophysiology 2011
Identification of a Novel Bacterial K+ Channel
G Tang, B Jiang, Y Huang, M Fu, L Wu, R Wang 		The Journal of Membrane Biology 2011
Hyperinsulinaemic hypoglycaemia and diabetes mellitus due to dominant ABCC8/KCNJ11 mutations
RR Kapoor, SE Flanagan, CT James, J McKiernan, AM Thomas, SC Harmer, JP Shield, A Tinker, S Ellard, K Hussain 		Diabetologia 2011
Current understanding of KATP channels in neonatal diseases: focus on insulin secretion disorders
Y Quan, A Barszczyk, Z Feng, H Sun 		Acta Pharmacologica Sinica 2011
Meta-analysis of genome-wide association studies identifies 8 new loci for type 2 diabetes in East Asians
Cho YS, Chen CH, Hu C, Long J, Ong RT, Sim X, Takeuchi F, Wu Y, Go MJ, Yamauchi T, Chang YC, Kwak SH, Ma RC, Yamamoto K, Adair LS, Aung T, Cai Q, Chang LC, Chen YT, Gao Y, Hu FB, Kim HL, Kim S, Kim YJ, Lee JJ, Lee NR, Li Y, Liu JJ, Lu W, Nakamura J, Nakashima E, Ng DP, Tay WT, Tsai FJ, Wong TY, Yokota M, Zheng W, Zhang R, Wang C, So WY, Ohnaka K, Ikegami H, Hara K, Cho YM, Cho NH, Chang TJ, Bao Y, Hedman ÅK, Morris AP, McCarthy MI, Takayanagi R, Park KS, Jia W, Chuang LM, Chan JC, Maeda S, Kadowaki T, Lee JY, Wu JY, Teo YY, Tai ES, Shu XO, Mohlke KL, Kato N, Han BG, Seielstad M 		Nature Genetics 2011
A role of the sulfonylurea receptor 1 in endocytic trafficking of ATP-sensitive potassium channels
Bruederle CE, Gay J, Shyng SL 		Traffic (Copenhagen, Denmark) 2011
N-terminal transmembrane domain of SUR1 controls gating of Kir6.2 by modulating channel sensitivity to PIP2
Pratt EB, Tewson P, Bruederle CE, Skach WR, Shyng SL 		The Journal of General Physiology 2011
Treatment with somatostatin analogs and chemoembolization of liver metastases for severe hypoglycemia in malignant insulinomas.
Maiza JC, Vezzosi D, Grunenwald S, Otal P, Guimbaud R, Bennet A, Caron P 		Journal of Endocrinological Investigation 2011
Diazoxide-responsive hyperinsulinemic hypoglycemia caused by HNF4A gene mutations
SE Flanagan, RR Kapoor, G Mali, D Cody, N Murphy, B Schwahn, T Siahanidou, I Banerjee, T Akcay, O Rubio-Cabezas, JP Shield, K Hussain, S Ellard 		European Journal of Endocrinology 2010
Muscle KATP channels: recent insights to energy sensing and myoprotection
TP Flagg, D Enkvetchakul, JC Koster, CG Nichols 		Physiological reviews 2010
Pancreatic beta-cell signaling: toward better understanding of diabetes and its treatment
S Seino, T Shibasaki, K Minami 		Proceedings of the Japan Academy. Series B, Physical and biological sciences 2010
Noble gas xenon is a novel adenosine triphosphate-sensitive potassium channel opener
C Bantel, M Maze, S Trapp 		Anesthesiology 2010
Loss of AMP-activated protein kinase alpha2 subunit in mouse beta-cells impairs glucose-stimulated insulin secretion and inhibits their sensitivity to hypoglycaemia
C Beall, K Piipari, H Al-Qassab, MA Smith, N Parker, D Carling, B Viollet, DJ Withers, ML Ashford 		Biochemical Journal 2010
The molecular mechanisms and pharmacotherapy of ATP-sensitive potassium channel gene mutations underlying neonatal diabetes
V Lang, PE Light 		Pharmacogenomics and Personalized Medicine 2010
KATPchannels process nucleotide signals in muscle thermogenic response
S Reyes, S Park, A Terzic, AE Alekseev 		Critical Reviews in Biochemistry and Molecular Biology 2010
Age-related memory impairments due to reduced blood glucose responses to epinephrine
KA Morris, Q Chang, EG Mohler, PE Gold 		Neurobiology of Aging 2010
The isolated pancreatic islet as a micro-organ and its transplantation to cure diabetes: Celebrating the legacy of Paul Lacy
S Misler 		Islets 2010
A mutation in KCNJ11 causing human hyperinsulinism (Y12X) results in a glucose intolerant phenotype in the mouse
Hugill A, Shimomura K, Ashcroft FM, Cox RD 		Diabetologia 2010
Resveratrol Potentiates Glucose-stimulated Insulin Secretion in INS-1E β-Cells and Human Islets through a SIRT1-dependent Mechanism
Vetterli L, Brun T, Giovannoni L, Bosco D, Maechler P 		The Journal of biological chemistry 2010
Direct and Specific Activation of Human Inward Rectifier K+ Channels by Membrane Phosphatidylinositol 4,5-Bisphosphate
D'Avanzo N, Cheng WW, Doyle DA, Nichols CG 		The Journal of biological chemistry 2010
Role of Hsp90 in Biogenesis of the β-Cell ATP-sensitive Potassium Channel Complex
Yan FF, Pratt EB, Chen PC, Wang F, Skach WR, David LL, Shyng SL 		Molecular biology of the cell 2010
Sarcolemmal ATP-sensitive K+ channels control energy expenditure determining body weight
Alekseev AE, Reyes S, Yamada S, Hodgson-Zingman DM, Sattiraju S, Zhu Z, Sierra A, Gerbin M, Coetzee WA, Goldhamer DJ, Terzic A, Zingman LV 		Cell Metabolism 2010
Pulmonary surfactant: an immunological perspective
ZC Chroneos, Z Sever-Chroneos, VL Shepherd 		Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology 2009
A novel hydroxyfuroic acid compound as an insulin receptor activator. Structure and activity relationship of a prenylindole moiety to insulin receptor activation
HJ Tsai, SY Chou 		Journal of Biomedical Science 2009
Human K(ATP) channelopathies: diseases of metabolic homeostasis
TM Olson, A Terzic 		Pflügers Archiv : European journal of physiology 2009
Neuronal preconditioning by inhalational anesthetics: evidence for the role of plasmalemmal adenosine triphosphate-sensitive potassium channels
C Bantel, M Maze, S Trapp 		Anesthesiology 2009
Molecular Biology of KATP Channels and Implications for Health and Disease
A Akrouh, SE Halcomb, CG Nichols, M Sala-Rabanal 		IUBMB Life 2009
KATP channel Kir6.2 E23K variant overrepresented in human heart failure is associated with impaired exercise stress response
S Reyes, S Park, BD Johnson, A Terzic, TM Olson 		Human Genetics 2009
Unifying concepts in stimulus-secretion coupling in endocrine cells and some implications for therapeutics
S Misler 		AJP Advances in Physiology Education 2009
Intolerant of glucose and gasping for oxygen
FM Gribble 		Nature Medicine 2009
Characterization and functional restoration of a potassium channel Kir6.2 pore mutation identified in congenital hyperinsulinism
JD Bushman, JW Gay, P Tewson, CA Stanley, SL Shyng 		The Journal of biological chemistry 2009
Sulfonylurea receptor 1 mutations that cause opposite insulin secretion defects with chemical chaperone exposure
EB Pratt, FF Yan, JW Gay, CA Stanley, SL Shyng 		The Journal of biological chemistry 2009
K(ATP) channelopathies in the pancreas
MS Remedi, JC Koster 		Pflügers Archiv : European journal of physiology 2009
Accounting for Near-Normal Glucose Sensitivity in Kir6.2[AAA] Transgenic Mice
K Tsaneva-Atanasova, A Sherman 		Biophysical Journal 2009
Interaction between mutations in the slide helix of Kir6.2 associated with neonatal diabetes and neurological symptoms
R Männikkö, C Jefferies, SE Flanagan, A Hattersley, S Ellard, FM Ashcroft 		Human Molecular Genetics 2009
An In-Frame Deletion in Kir6.2 (KCNJ11) Causing Neonatal Diabetes Reveals a Site of Interaction between Kir6.2 and SUR1
Craig TJ, Shimomura K, Holl RW, Flanagan SE, Ellard S, Ashcroft FM 		The Journal of clinical endocrinology and metabolism 2009
Rapid Regulation of KATP Channel Activity by 17β-Estradiol in Pancreatic β-Cells Involves the Estrogen Receptor β and the Atrial Natriuretic Peptide Receptor
Soriano S, Ropero AB, Alonso-Magdalena P, Ripoll C, Quesada I, Gassner B, Kuhn M, Gustafsson JÅ, Nadal A 		Molecular Endocrinology 2009
Constitutive Endocytic Recycling and Protein Kinase C-mediated Lysosomal Degradation Control KATP Channel Surface Density
Manna PT, Smith AJ, Taneja TK, Howell GJ, Lippiat JD, Sivaprasadarao A 		The Journal of biological chemistry 2009
ATP-Sensitive K+ Channel Knockout Induces Cardiac Proteome Remodeling Predictive of Heart Disease Susceptibility
Arrell DK, Zlatkovic J, Kane GC, Yamada S, Terzic A 		Journal of Proteome Research 2009
QUATERNARY STRUCTURE OF KATP CHANNEL SUR2A NUCLEOTIDE BINDING DOMAINS RESOLVED BY SYNCHROTRON RADIATION X-RAY SCATTERING
Park S, Terzic A 		Journal of Structural Biology 2009
Glyburide inhibits the Cryopyrin/Nalp3 inflammasome
Lamkanfi M, Mueller JL, Vitari AC, Misaghi S, Fedorova A, Deshayes K, Lee WP, Hoffman HM, Dixit VM 		The Journal of Cell Biology 2009
Mitochondrial Glutamate Carrier GC1 as a Newly Identified Player in the Control of Glucose-stimulated Insulin Secretion
Casimir M, Lasorsa FM, Rubi B, Caille D, Palmieri F, Meda P, Maechler P 		The Journal of biological chemistry 2009
Visualization of ATP levels inside single living cells with fluorescence resonance energy transfer-based genetically encoded indicators
Imamura H, Huynh Nhat KP, Togawa H, Saito K, Iino R, Kato-Yamada Y, Nagai T, Noji H 		Proceedings of the National Academy of Sciences 2009
Impaired Insulin Exocytosis in Neural Cell Adhesion Molecule−/− Mice Due to Defective Reorganization of the Submembrane F-Actin Network
Olofsson CS, Håkansson J, Salehi A, Bengtsson M, Galvanovskis J, Partridge C, SörhedeWinzell M, Xian X, Eliasson L, Lundquist I, Semb H, Rorsman P 		Endocrinology 2009
KirBac1.1: It's an Inward Rectifying Potassium Channel
Cheng WW, Enkvetchakul D, Nichols CG 		The Journal of General Physiology 2009
Analysis of Two KCNJ11 Neonatal Diabetes Mutations, V59G and V59A, and the Analogous KCNJ8 I60G Substitution: DIFFERENCES BETWEEN THE CHANNEL SUBTYPES FORMED WITH SUR1
Winkler M, Lutz R, Russ U, Quast U, Bryan J 		The Journal of biological chemistry 2009
KCNJ11 knockout morula re-engineered by stem cell diploid aggregation
TJ Nelson, A Martinez-Fernandez, A Terzic 		Philosophical transactions of the Royal Society of London. Series B, Biological sciences 2008
Molecular mechanisms and clinical implications of reversible protein S-glutathionylation
JJ Mieyal, MM Gallogly, S Qanungo, EA Sabens, MD Shelton 		Antioxidants & Redox Signaling 2008
K + Channel Modulators for the Treatment of Neurological Disorders and Autoimmune Diseases
H Wulff, BS Zhorov 		Chemical Reviews 2008
Ameliorating Effects of Sulfonylurea Drugs on Insulin Resistance in Otsuka Long-Evans Tokushima Fatty Rats
JK Park, SP Kim, DK Song 		The Korean Journal of Physiology and Pharmacology 2008
A rare mutation in ABCC8/SUR1 leading to altered KATP channel activity and β-cell glucose sensing is associated with type 2 diabetes mellitus in adults
Tarasov AI, Nicolson T, Riveline JP, Taneja TK, Baldwin SA, Baldwin JM, Charpentier G, Gautier JF, Froguel P, Vaxillaire M, Rutter GA 		Diabetes 2008
Interaction of Asymmetric ABCC9-Encoded Nucleotide Binding Domains Determines KATP Channel SUR2A Catalytic Activity
Park S, Lim BB, Perez-Terzic C, Mer G, Terzic A 		Journal of Proteome Research 2008
SUR1: a unique ATP-binding cassette protein that functions as an ion channel regulator
Aittoniemi J, Fotinou C, Craig TJ, de Wet H, Proks P, Ashcroft FM 		Philosophical Transactions of the Royal Society B: Biological Sciences 2008
Functional Clustering of Mutations in the Dimer Interface of the Nucleotide Binding Folds of the Sulfonylurea Receptor
Masia R, Nichols CG 		The Journal of biological chemistry 2008
Coassembly of Different Sulfonylurea Receptor Subtypes Extends the Phenotypic Diversity of ATP-sensitive Potassium (KATP) Channels
Wheeler A, Wang C, Yang K, Fang K, Davis K, Styer AM, Mirshahi U, Moreau C, Revilloud J, Vivaudou M, Liu S, Mirshahi T, Chan KW 		Molecular pharmacology 2008
How ATP Inhibits the Open KATP Channel
Craig TJ, Ashcroft FM, Proks P 		The Journal of General Physiology 2008
Destabilization of ATP-sensitive Potassium Channel Activity by Novel KCNJ11 Mutations Identified in Congenital Hyperinsulinism
Lin YW, Bushman JD, Yan FF, Haidar S, MacMullen C, Ganguly A, Stanley CA, Shyng SL 		The Journal of biological chemistry 2008
Role of the cAMP sensor Epac as a determinant of KATP channel ATP sensitivity in human pancreatic β-cells and rat INS-1 cells
Kang G, Leech CA, Chepurny OG, Coetzee WA, Holz GG 		The Journal of Physiology 2008
A Kir6.2 mutation causing severe functional effects in vitro produces neonatal diabetes without the expected neurological complications
Tammaro P, Flanagan SE, Zadek B, Srinivasan S, Woodhead H, Hameed S, Klimes I, Hattersley AT, Ellard S, Ashcroft FM 		Diabetologia 2008
Sulfonylurea Receptors Type 1 and 2A Randomly Assemble to Form Heteromeric KATP Channels of Mixed Subunit Composition
Chan KW, Wheeler A, Csanády L 		The Journal of General Physiology 2008
Dysglycaemias and fluoroquinolones.
Lewis RJ, Mohr JF 3rd 		Drug Safety 2008
A K ATP channel-dependent pathway within alpha cells regulates glucagon release from both rodent and human islets of Langerhans
PE MacDonald, YZ de Marinis, R Ramracheya, A Salehi, X Ma, PR Johnson, R Cox, L Eliasson, P Rorsman 		PLoS Biology 2007
K(ATP) channel pharmacogenomics: from bench to bedside
S Sattiraju, S Reyes, GC Kane, A Terzic 		Clinical Pharmacology & Therapeutics 2007
Prevalence of Permanent Neonatal Diabetes in Slovakia and Successful Replacement of Insulin with Sulfonylurea Therapy in KCNJ11 and ABCC8 Mutation Carriers
Stanik J, Gasperikova D, Paskova M, Barak L, Javorkova J, Jancova E, Ciljakova M, Hlava P, Michalek J, Flanagan SE, Pearson E, Hattersley AT, Ellard S, Klimes I 		The Journal of clinical endocrinology and metabolism 2007
A mutation in the ATP-binding site of the Kir6.2 subunit of the KATP channel alters coupling with the SUR2A subunit
Tammaro P, Ashcroft FM 		The Journal of Physiology 2007
Is Kir6.1 a subunit of mitoKATP?
Foster DB, Rucker JJ, Marbán E 		Biochemical and Biophysical Research Communications 2007
Congenital Hyperinsulinism–Associated ABCC8 Mutations That Cause Defective Trafficking of ATP-Sensitive K+ Channels: Identification and Rescue
Yan FF, Lin YW, MacMullen C, Ganguly A, Stanley CA, Shyng SL 		Diabetes 2007
Increased ATPase activity produced by mutations at arginine-1380 in nucleotide-binding domain 2 of ABCC8 causes neonatal diabetes
de Wet H, Rees MG, Shimomura K, Aittoniemi J, Patch AM, Flanagan SE, Ellard S, Hattersley AT, Sansom MS, Ashcroft FM 		Proceedings of the National Academy of Sciences 2007
KATP channel mutation confers risk for vein of Marshall adrenergic atrial fibrillation
Olson TM, Alekseev AE, Moreau C, Liu XK, Zingman LV, Miki T, Seino S, Asirvatham SJ, Jahangir A, Terzic A 		Nature clinical practice. Cardiovascular medicine 2007
The Kir6.2-F333I mutation differentially modulates KATP channels composed of SUR1 or SUR2 subunits
Tammaro P, Ashcroft F 		The Journal of Physiology 2007
Identification of the PIP2-binding site on Kir6.2 by molecular modelling and functional analysis
Haider S, Tarasov AI, Craig TJ, Sansom MS, Ashcroft FM 		The EMBO Journal 2007
Central control of glucose homeostasis
A Puglianiello, S Cianfarani 		The review of diabetic studies : RDS 2006
Central nervous system and control of endogenous glucose production
G Demuro, S Obici 		Current Diabetes Reports 2006
Functional effects of naturally occurring KCNJ11 mutations causing neonatal diabetes on cloned cardiac K ATP channels: Cardiac K ATP channels carrying human Kir6.2 mutations
P Tammaro, P Proks, FM Ashcroft 		The Journal of Physiology 2006
Protection conferred by myocardial ATP-sensitive K + channels in pressure overload-induced congestive heart failure revealed in KCNJ11 Kir6.2-null mutant: Kir6.2 knockout precipitates heart failure
S Yamada, GC Kane, A Behfar, XK Liu, RB Dyer, RS Faustino, T Miki, S Seino, A Terzic 		The Journal of Physiology 2006
K+ Channels in Apoptosis
ED Burg, CV Remillard, JX Yuan 		The Journal of Membrane Biology 2006
Pancreatic Islets from Hypothalamic Obese Rats Maintain K+ATP Channel-Dependent but Not -Independent Pathways on Glucose-Induced Insulin Release Process
S Grassiolli, ML Bonfleur, DX Scomparin, PC de Freitas Mathias 		Endocrine 2006
The N-terminal transmembrane domain (TMD0) and a cytosolic linker (L0) of sulphonylurea receptor define the unique intrinsic gating of KATP channels
Fang K, Csanády L, Chan KW 		The Journal of Physiology 2006
cAMP sensor Epac as a determinant of ATP-sensitive potassium channel activity in human pancreatic β cells and rat INS-1 cells
Kang G, Chepurny OG, Malester B, Rindler MJ, Rehmann H, Bos JL, Schwede F, Coetzee WA, Holz GG 		The Journal of Physiology 2006
Glibenclamide, a Blocker of K+ATP Channels, Shows Antileishmanial Activity in Experimental Murine Cutaneous Leishmaniasis
Serrano-Martín X, Payares G, Mendoza-León A 		Antimicrobial agents and chemotherapy 2006
Kir6.2 mutations causing neonatal diabetes prevent endocytosis of ATP-sensitive potassium channels
Mankouri J, Taneja TK, Smith AJ, Ponnambalam S, Sivaprasadarao A 		The EMBO Journal 2006
Structural and functional analysis of the putative pH sensor in the Kir1.1 (ROMK) potassium channel
Rapedius M, Haider S, Browne KF, Shang L, Sansom MS, Baukrowitz T, Tucker SJ 		EMBO reports 2006
Functional analysis of six Kir6.2 (KCNJ11) mutations causing neonatal diabetes.
Girard CA, Shimomura K, Proks P, Absalom N, Castano L, Perez de Nanclares G, Ashcroft FM 		2006
The channelopathies: novel insights into molecular and genetic mechanisms of human disease
RS Kass 		Journal of Clinical Investigation 2005
3-D structural and functional characterization of the purified KATP channel complex Kir6.2–SUR1
Mikhailov MV, Campbell JD, de Wet H, Shimomura K, Zadek B, Collins RF, Sansom MS, Ford RC, Ashcroft FM 		The EMBO Journal 2005

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