Abcc8

Summary

Gene Symbol: Abcc8
Description: ATP-binding cassette, sub-family C (CFTR/MRP), member 8
Alias: D930031B21Rik, SUR1, Sur, ATP-binding cassette sub-family C member 8, sulfonylurea receptor
Species: mouse

Top Publications

  1. pmc Mice transgenically overexpressing sulfonylurea receptor 1 in forebrain resist seizure induction and excitotoxic neuron death
    C Hernandez-Sanchez
    Section on Molecular and Cellular Physiology, Clinical Endocrinology Branch, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 98:3549-54. 2001
  2. ncbi Transactivation of the mouse sulfonylurea receptor I gene by BETA2/NeuroD
    Ji Won Kim
    Department of Anatomy, Ajou University, School of Medicine, Suwon, 442 749, Korea
    Mol Endocrinol 16:1097-107. 2002
  3. ncbi ATP-sensitive K+ channel-dependent regulation of glucagon release and electrical activity by glucose in wild-type and SUR1-/- mouse alpha-cells
    Jesper Gromada
    Lilly Research Laboratories, Essener Strasse 93, D 22419 Hamburg, Germany
    Diabetes 53:S181-9. 2004
  4. pmc Exendin-(9-39) corrects fasting hypoglycemia in SUR-1-/- mice by lowering cAMP in pancreatic beta-cells and inhibiting insulin secretion
    Diva D De Leon
    Division of Endocrinology, Department of Pediatrics, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 283:25786-93. 2008
  5. pmc Differential structure of atrial and ventricular KATP: atrial KATP channels require SUR1
    Thomas P Flagg
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, MO 63110, USA
    Circ Res 103:1458-65. 2008
  6. pmc Differential K(ATP) channel pharmacology in intact mouse heart
    Alexey V Glukhov
    Department of Biomedical Engineering, Washington University, St Louis, MO 63130, USA
    J Mol Cell Cardiol 48:152-60. 2010
  7. pmc ATP-sensitive K+ channel mediates the zinc switch-off signal for glucagon response during glucose deprivation
    Michela Slucca
    Pacific Northwest Diabetes Research Institute and the Division of Metabolism, Endocrinology, and Nutrition, Department of Medicine and Department of Pharmacology, University of Washington, Seattle, Washington, USA
    Diabetes 59:128-34. 2010
  8. ncbi cAMP-activated protein kinase-independent potentiation of insulin secretion by cAMP is impaired in SUR1 null islets
    Mitsuhiro Nakazaki
    Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, TX 77030, USA
    Diabetes 51:3440-9. 2002
  9. ncbi Sulfonylurea receptor type 1 knock-out mice have intact feeding-stimulated insulin secretion despite marked impairment in their response to glucose
    Chiyo Shiota
    Department of Molecular Physiology and Biophysics, Vanderbilt University School of Medicine, Nashville, Tennessee 37232, USA
    J Biol Chem 277:37176-83. 2002
  10. ncbi Overnight culture unmasks glucose-induced insulin secretion in mouse islets lacking ATP-sensitive K+ channels by improving the triggering Ca2+ signal
    Andras Szollosi
    Unit of Endocrinology and Metabolism, University of Louvain Faculty of Medicine, UCL55 30, B 1200 Brussels, Belgium
    J Biol Chem 282:14768-76. 2007

Scientific Experts

  • Thomas P Flagg
  • S Andrikopoulos
  • CHRISTOPHER JOHN LYNCH
  • Annette Hambrock
  • J Marc Simard
  • P E Squires
  • Maria Remedi
  • Linda Ernst
  • B Breant
  • J Gromada
  • Joseph Bryan
  • DIVA DE LEON
  • Lydia Aguilar-Bryan
  • Colin G Nichols
  • Changhong Li
  • Alexey V Glukhov
  • Andras Szollosi
  • Franz M Matschinsky
  • Ihsane Marhfour
  • Mark A Magnuson
  • Alvaro Munoz
  • Mitsuhiro Nakazaki
  • Myriam Nenquin
  • Heather W Collins
  • Nicolai M Doliba
  • Chiyo Shiota
  • C Hernandez-Sanchez
  • Mengyin Cai
  • Charles A Stanley
  • Markku Laakso
  • L Aguilar-Bryan
  • Jean Claude Henquin
  • Kunpeng Zhao
  • Kenju Shimomura
  • Rui Cheng-Xue
  • Tony K T Lam
  • Alessandro Pocai
  • Naheed Fatima
  • Monica Sala-Rabanal
  • D Haspel
  • Chutima Talchai
  • J Bryan
  • M Dufer
  • Lena Eliasson
  • Igor R Efimov
  • B Valtat
  • Youhou Kang
  • Karen K Yee
  • Yu Fukuda
  • Per Olof Berggren
  • Xuehui Geng
  • Mayrin C Medina
  • YouMing Lu
  • Dong Kong
  • Michela Slucca
  • Eric Hosy
  • Frances M Ashcroft
  • Angela D Sellitto
  • Christine Sempoux
  • Yves Guiot
  • Jacques Rahier
  • Joelle Marchandise
  • Stefanie Ackermann
  • Belinda Gier
  • Diva D De Leon
  • Wenyu Huang
  • Xinli Hu
  • John W Elrod
  • Victor Seghers
  • Claudia Miele
  • Y Guiot
  • Quan Zhang
  • Mangala M Soundarapandian
  • Show Ling Shyng
  • William A Coetzee
  • Susanne Korff
  • Yevgeny Daikhin
  • Habiba Najafi
  • Marc Yudkoff
  • A Varadi
  • Carol Buettger
  • Carol W Buettger
  • Patrik Rorsman
  • Erik Renstrom
  • Richard D Carr
  • Luciano Rossetti
  • Alison Morrissey
  • Marko Z Vatamaniuk
  • Albert Salehi
  • Gary J Schwartz

Detail Information

Publications76

  1. pmc Mice transgenically overexpressing sulfonylurea receptor 1 in forebrain resist seizure induction and excitotoxic neuron death
    C Hernandez-Sanchez
    Section on Molecular and Cellular Physiology, Clinical Endocrinology Branch, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 98:3549-54. 2001
    The ability of the sulfonylurea receptor (SUR) 1 to suppress seizures and excitotoxic neuron damage was assessed in mice transgenically overexpressing this receptor...
  2. ncbi Transactivation of the mouse sulfonylurea receptor I gene by BETA2/NeuroD
    Ji Won Kim
    Department of Anatomy, Ajou University, School of Medicine, Suwon, 442 749, Korea
    Mol Endocrinol 16:1097-107. 2002
    The sulfonylurea receptor 1 (SUR1) plays a key role in regulation of insulin secretion in pancreatic beta-cells. In this study we investigated the mechanism for tissue-specific expression of the SUR1 gene...
  3. ncbi ATP-sensitive K+ channel-dependent regulation of glucagon release and electrical activity by glucose in wild-type and SUR1-/- mouse alpha-cells
    Jesper Gromada
    Lilly Research Laboratories, Essener Strasse 93, D 22419 Hamburg, Germany
    Diabetes 53:S181-9. 2004
    ..In mice lacking functional KATP channels (SUR1-/-), glucagon secretion in the absence of glucose was lower than that observed in wild-type islets and both glucose ..
  4. pmc Exendin-(9-39) corrects fasting hypoglycemia in SUR-1-/- mice by lowering cAMP in pancreatic beta-cells and inhibiting insulin secretion
    Diva D De Leon
    Division of Endocrinology, Department of Pediatrics, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 283:25786-93. 2008
    ..Loss-of-function mutations in the K(ATP) channel (composed of two subunits: Kir6.2 and SUR-1) are responsible for the most common and severe form of congenital hyperinsulinism...
  5. pmc Differential structure of atrial and ventricular KATP: atrial KATP channels require SUR1
    Thomas P Flagg
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, MO 63110, USA
    Circ Res 103:1458-65. 2008
    ..Using a novel antibody directed against the COOH terminus of SUR1 (ABCC8), we show that this K(ATP) subunit is also expressed in mouse myocardium and is the dominant SUR isoform in the ..
  6. pmc Differential K(ATP) channel pharmacology in intact mouse heart
    Alexey V Glukhov
    Department of Biomedical Engineering, Washington University, St Louis, MO 63130, USA
    J Mol Cell Cardiol 48:152-60. 2010
    ..2 (KCNJ11) and SUR2A (ABCC9) subunits. However, the evidence is strong that SUR1 (sulfonylurea receptor type 1, ABCC8) subunits are also expressed in the heart and that they play a significant functional role ..
  7. pmc ATP-sensitive K+ channel mediates the zinc switch-off signal for glucagon response during glucose deprivation
    Michela Slucca
    Pacific Northwest Diabetes Research Institute and the Division of Metabolism, Endocrinology, and Nutrition, Department of Medicine and Department of Pharmacology, University of Washington, Seattle, Washington, USA
    Diabetes 59:128-34. 2010
    ..These studies were designed to determine whether closure of the alpha-cell ATP-sensitive K(+) channel (K(ATP) channel) is the mechanism through which the zinc switch-off signal triggers glucagon secretion during glucose deprivation...
  8. ncbi cAMP-activated protein kinase-independent potentiation of insulin secretion by cAMP is impaired in SUR1 null islets
    Mitsuhiro Nakazaki
    Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, TX 77030, USA
    Diabetes 51:3440-9. 2002
    ..causes severe hypoglycemia in certain forms of hyperinsulinemic hypoglycemia, similar channel loss in sulfonylurea receptor-1 (SUR1) and Kir6...
  9. ncbi Sulfonylurea receptor type 1 knock-out mice have intact feeding-stimulated insulin secretion despite marked impairment in their response to glucose
    Chiyo Shiota
    Department of Molecular Physiology and Biophysics, Vanderbilt University School of Medicine, Nashville, Tennessee 37232, USA
    J Biol Chem 277:37176-83. 2002
    ..In humans, mutations in either of the two subunits for this channel, the sulfonylurea type 1 receptor (Sur1) or Kir6.2, cause persistent hyperinsulinemic hypoglycemia of infancy...
  10. ncbi Overnight culture unmasks glucose-induced insulin secretion in mouse islets lacking ATP-sensitive K+ channels by improving the triggering Ca2+ signal
    Andras Szollosi
    Unit of Endocrinology and Metabolism, University of Louvain Faculty of Medicine, UCL55 30, B 1200 Brussels, Belgium
    J Biol Chem 282:14768-76. 2007
    ..However, several studies of sulfonylurea receptor 1 null mice (Sur1KO) failed to measure significant effects of glucose in their islets lacking K(ATP) ..
  11. ncbi Both triggering and amplifying pathways contribute to fuel-induced insulin secretion in the absence of sulfonylurea receptor-1 in pancreatic beta-cells
    Myriam Nenquin
    Unité d Endocrinologie et Métabolisme, University of Louvain Faculty of Medicine UCL 55 30, Ave Hippocrate 55, B 1200 Brussels, Belgium
    J Biol Chem 279:32316-24. 2004
    ..It is unclear if and how nutrients can regulate insulin secretion by beta-cells lacking K(ATP) channels (Sur1 knockout mice)...
  12. ncbi Sur1 knockout mice. A model for K(ATP) channel-independent regulation of insulin secretion
    V Seghers
    Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 275:9270-7. 2000
    b>Sur1 knockout mouse beta-cells lack K(ATP) channels and show spontaneous Ca(2+) action potentials equivalent to those seen in patients with persistent hyperinsulinemic hypoglycemia of infancy, but the mice are normoglycemic unless ..
  13. ncbi Hypothalamic sensing of circulating fatty acids is required for glucose homeostasis
    Tony K T Lam
    Department of Medicine, Diabetes Research Center, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Belfer 701, Bronx, New York 10461, USA
    Nat Med 11:320-7. 2005
    ..glucose fluxes through (i) inhibition of the hypothalamic esterification of fatty acids, (ii) genetic deletion (Sur1-deficient mice) of hypothalamic K(ATP) channels or pharmacological blockade (K(ATP) blocker) of their activation by ..
  14. ncbi Characterization of the mouse sulfonylurea receptor 1 promoter and its regulation
    C Hernandez-Sanchez
    Section on Molecular and Cellular Physiology, Diabetes Branch, NIDDK, National Institutes of Health, Bethesda, Maryland 20892 1770, USA
    J Biol Chem 274:18261-70. 1999
    ..potassium channels (K+ATP channels) are heteromultimeric structures formed by a member of the sulfonylurea receptor (SUR) family and a member of the inwardly rectifying potassium channel family (Kir6.x)...
  15. pmc Tolbutamide controls glucagon release from mouse islets differently than glucose: involvement of K(ATP) channels from both α-cells and δ-cells
    Rui Cheng-Xue
    pôle d endocrinologie, diabète et nutrition, Institut de Recherche Expérimentale et Clinique, Universite Catholique de Louvain, Brussels, Belgium
    Diabetes 62:1612-22. 2013
    ..glucagon secretion produced by high concentrations of tolbutamide or diazoxide, or disruption of K(ATP) channels (Sur1(-/-) mice) at G1 could be inhibited further by G7...
  16. ncbi Glucose stimulates Ca2+ influx and insulin secretion in 2-week-old beta-cells lacking ATP-sensitive K+ channels
    Andras Szollosi
    Unit of Endocrinology and Metabolism, University of Louvain Faculty of Medicine, UCL 55 30, Avenue Hippocrate 55, B 1200 Brussels, Belgium
    J Biol Chem 282:1747-56. 2007
    ..Mice lacking the high affinity sulfonylurea receptor (Sur1KO), and thus K(ATP) channels, have been developed as a model of congenital hyperinsulinism...
  17. ncbi Hyperinsulinism in mice with heterozygous loss of K(ATP) channels
    M S Remedi
    Department of Cell Biology and Physiology, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Diabetologia 49:2368-78. 2006
    ..In humans, loss-of-function mutations of beta cell K(ATP) subunits (SUR1, encoded by the gene ABCC8, or Kir6.2, encoded by the gene KCNJ11) cause congenital hyperinsulinaemia...
  18. ncbi Hypothalamic K(ATP) channels control hepatic glucose production
    Alessandro Pocai
    Department of Medicine, Diabetes Research Center, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    Nature 434:1026-31. 2005
    ..Consistent with these results, mice lacking the SUR1 subunit of the K(ATP) channel are resistant to the inhibitory action of insulin on gluconeogenesis...
  19. ncbi Impaired glucagon secretory responses in mice lacking the type 1 sulfonylurea receptor
    Chiyo Shiota
    Vanderbilt University School of Medicine, Dept of Molecular Physiology and Biophysics, Nashville, TN 37232 0615, USA
    Am J Physiol Endocrinol Metab 289:E570-7. 2005
    ..role of K(ATP) channels in alpha-cells, we examined glucagon secretion in mice lacking the type 1 sulfonylurea receptor (Sur1)...
  20. ncbi Regulation of glucagon secretion at low glucose concentrations: evidence for adenosine triphosphate-sensitive potassium channel involvement
    Alvaro Munoz
    Departments of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA
    Endocrinology 146:5514-21. 2005
    ..b>sur1/k(ir)6...
  21. doi Epigenetic regulation of glucose-stimulated osteopontin (OPN) expression in diabetic kidney
    Mengyin Cai
    Diabetes and Endocrinology, Department of Clinical Sciences, Skåne University Hospital Malmö, Lund University, 20502 Malmo, Sweden Department of Endocrinology, The Third Affiliated Hospital of Sun Yat Sen University, 510630 Guangzhou, China
    Biochem Biophys Res Commun 469:108-13. 2016
    ..we demonstrated that OPN gene expression is upregulated in the kidneys of a hyperglycemia diabetes mouse model SUR1-E1506K, and glucose-stimulated OPN gene expression is strongly associated with increases in activating histone ..
  22. ncbi Expression of functional Kir6.1 channels regulates glutamate release at CA3 synapses in generation of epileptic form of seizures
    Mangala M Soundarapandian
    Biomedical Science Center, Burnett College of Biomedical Sciences, University of Central Florida, Orlando, Florida, USA
    J Neurochem 103:1982-8. 2007
    ..1 channels are located at excitatory pre-synaptic terminals as a complex with type-1 Sulfonylurea receptors (SUR1) in the hippocampus. The mutant mice with deficiencies in expressing the Kir6...
  23. pmc Functional roles of KATP channel subunits in metabolic inhibition
    Alexey V Glukhov
    Department of Biomedical Engineering, Washington University, St Louis, MO 63130, USA
    J Mol Cell Cardiol 62:90-8. 2013
    ..We showed previously that SUR1 with Kir6.2 forms the functional channel in mouse atria while Kir6.2 and SUR2A predominate in ventricles...
  24. pmc A mouse model of human hyperinsulinism produced by the E1506K mutation in the sulphonylurea receptor SUR1
    Kenju Shimomura
    Henry Wellcome Centre for Gene Function, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, U K
    Diabetes 62:3797-806. 2013
    Loss-of-function mutations in the KATP channel genes KCNJ11 and ABCC8 cause neonatal hyperinsulinism in humans...
  25. doi Identification of ABCC8 as a contributory gene to impaired early-phase insulin secretion in NZO mice
    Sofianos Andrikopoulos
    Department of Medicine AH Austin Hospital, University of Melbourne, Level 7, Lance Townsend Building, Studley Road, Heidelberg, Victoria 3084, AustraliaWalter and Eliza Hall Institute of Medical ResearchParkville, 3010 Victoria, AustraliaHarry Perkins Institute of Medical ResearchNedlands, Western Australia 6009, AustraliaThe School of Medical Sciences Edith Cowan UniversityJoondalup Western Australia 6027, Australia
    J Endocrinol 228:61-73. 2016
    ..Using congenic strains, the locus was narrowed to two candidate genes encoding the components of the KATP channel: Abcc8 (SUR1) and Kcnj11 (Kir6.2)...
  26. pmc α-Synuclein binds the K(ATP) channel at insulin-secretory granules and inhibits insulin secretion
    Xuehui Geng
    Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pennsylvania, USA
    Am J Physiol Endocrinol Metab 300:E276-86. 2011
    ..Synuclein might play similar roles in diabetes as it does in other degenerative diseases, including Alzheimer's and Parkinson's diseases...
  27. pmc Some cannabinoid receptor ligands and their distomers are direct-acting openers of SUR1 K(ATP) channels
    Christopher J Lynch
    Dept of Cellular and Molecular Physiology, Pennsylvania State College of Medicine, Hershey, PA 17033, USA
    Am J Physiol Endocrinol Metab 302:E540-51. 2012
    ..In vivo rimonabant and ibipinabant caused glucose intolerance in CB1 but not SUR1-KO mice...
  28. pmc On potential interactions between non-selective cation channel TRPM4 and sulfonylurea receptor SUR1
    Monica Sala-Rabanal
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 287:8746-56. 2012
    The sulfonylurea receptor SUR1 associates with Kir6.2 or Kir6.1 to form K(ATP) channels, which link metabolism to excitability in multiple cell types...
  29. pmc Generation of functional insulin-producing cells in the gut by Foxo1 ablation
    Chutima Talchai
    Naomi Berrie Diabetes Center, Columbia University Medical Center, New York, New York, USA
    Nat Genet 44:406-12, S1. 2012
    ..The data indicate that Neurog3(+) enteroendocrine progenitors require active Foxo1 to prevent differentiation into Ins(+) cells. Foxo1 ablation in gut epithelium may provide an approach to restore insulin production in type 1 diabetes...
  30. pmc Promoter DNA methylation regulates murine SUR1 (Abcc8) and SUR2 (Abcc9) expression in HL-1 cardiomyocytes
    Naheed Fatima
    Department of Anatomy, Physiology and Genetics, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine, Bethesda, Maryland, United States of America
    PLoS ONE 7:e41533. 2012
    Two mammalian genes encode the SURx (SUR1, Abcc8 and SUR2, Abcc9) subunits that combine with Kir6.2 (Kcnj11) subunits to form the ATP-sensitive potassium (KATP) channel in cardiac myocytes...
  31. ncbi R-type Ca(2+)-channel-evoked CICR regulates glucose-induced somatostatin secretion
    Quan Zhang
    Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Churchill Hospital, Oxford OX3 7LJ, UK
    Nat Cell Biol 9:453-60. 2007
    ..Glucose-induced somatostatin secretion is instead primarily dependent on Ca(2+)-induced Ca(2+)-release (CICR). This constitutes a novel mechanism for K(ATP)-channel-independent metabolic control of pancreatic hormone secretion...
  32. pmc EPAC inhibition of SUR1 receptor increases glutamate release and seizure vulnerability
    Kunpeng Zhao
    Key Laboratory of Neurological Disease, Ministry of Education, Huazhong University of Science and Technology Tongji Medical College, Wuhan, 430030 China
    J Neurosci 33:8861-5. 2013
    ..that EPAC binds directly to the intracellular loop of an ATP-sensitive potassium (KATP) channel type-1 sulfonylurea receptor (SUR1) receptor consisting of amino acids 859-881 (SUR1(859-881))...
  33. ncbi A novel sulfonylurea receptor forms with BIR (Kir6.2) a smooth muscle type ATP-sensitive K+ channel
    S Isomoto
    Department of Pharmacology II, Faculty of Medicine, Osaka University, 2 2 Yamadaoka, Suita, Osaka 565, Japan
    J Biol Chem 271:24321-4. 1996
    We have isolated a cDNA encoding a novel isoform of the sulfonylurea receptor from a mouse heart cDNA library. Coexpression of this isoform and BIR (Kir6.2) in a mammalian cell line elicited ATP-sensitive K+ (KATP) channel currents...
  34. ncbi Oscillations of membrane potential and cytosolic Ca(2+) concentration in SUR1(-/-) beta cells
    M Dufer
    Department of Pharmacology, University of Tubingen, Auf der Morgenstelle 8, 72076, Tubingen, Germany
    Diabetologia 47:488-98. 2004
    SUR1(ABCC8)(-/-) mice lacking functional K(ATP) channels are an appropriate model to test the significance of K(ATP) channels in beta-cell function. We examined how this gene deletion interferes with stimulus-secretion coupling...
  35. pmc Sulfonylurea receptor knockout causes glucose intolerance in mice that is not alleviated by concomitant somatostatin subtype receptor 5 knockout
    Michael Norman
    Michael E DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas 77030, USA
    Ann Surg 235:767-74. 2002
    To examine the long-term effects of Sur KO, SSTR5 KO, and double Sur/SSTR5 KO on insulin secretion and glucose regulation.
  36. ncbi Ischemic preconditioning in the hippocampus of a knockout mouse lacking SUR1-based K(ATP) channels
    Alvaro Munoz
    Department of Medicine, Baylor College of Medicine, Houston, Tex 77030, USA
    Stroke 34:164-70. 2003
    ..To evaluate the role of neuronal/beta-cell-type K(ATP) channels, SUR1 null (Sur1KO) mice lacking (K(IR)6...
  37. pmc Suppression of KATP channel activity protects murine pancreatic beta cells against oxidative stress
    Belinda Gier
    Institute of Pharmacy, Department of Pharmacology, University of Tubingen, Tubingen, Germany
    J Clin Invest 119:3246-56. 2009
    ..Using mice lacking the sulfonylurea receptor type 1 (Sur1) subunit of KATP channels, we found that, compared with insulin secretion by WT islets, ..
  38. pmc Impact of disease-causing SUR1 mutations on the KATP channel subunit interface probed with a rhodamine protection assay
    Eric Hosy
    Institut de Biologie Structurale CEA, CNRS, UJF, Laboratoire des Proteines Membranaires, 41 rue Jules Horowitz, 38027 Grenoble, France
    J Biol Chem 285:3084-91. 2010
    ..The activating mutation F132L in SUR1, which causes neonatal diabetes, also rendered the channel resistant to Rho block, suggesting that it stabilized an ..
  39. doi Endoplasmic reticulum accumulation of Kir6.2 without activation of ER stress response in islet cells from adult Sur1 knockout mice
    Ihsane Marhfour
    Department of Pathology, Cliniques Universitaires Saint Luc, Universite Catholique de Louvain, Tour Rosalind Franklin, 10 Avenue Hippocrate, 1200, Brussels, Belgium
    Cell Tissue Res 340:335-46. 2010
    ..to the plasma membrane critically depends on masking the endoplasmic reticulum (ER) retention signals of the SUR1 and Kir6.2 subunits upon their proper assembly into functional hetero-octamers...
  40. pmc Brief suppression of Abcc8 prevents autodestruction of spinal cord after trauma
    J Marc Simard
    Department of Neurosurgery, University of Maryland School of Medicine, 22 South Greene Street, Suite S12D, Baltimore, MD 21201 1595, USA
    Sci Transl Med 2:28ra29. 2010
    ..We assessed the role of Abcc8, which encodes sulfonylurea receptor 1 (SUR1), in progressive hemorrhagic necrosis...
  41. pmc Diazoxide maintenance of myocyte volume and contractility during stress: evidence for a non-sarcolemmal K(ATP) channel location
    Angela D Sellitto
    Division of Cardiothoracic Surgery, Department of Surgery, Washington University School of Medicine, St Louis, MO 63110, USA
    J Thorac Cardiovasc Surg 140:1153-9. 2010
    ..Both SUR1 and SUR2A subunits have been localized to the heart, and mouse sarcolemmal adenosine triphosphate-sensitive ..
  42. doi Genetic evidence of the programming of beta cell mass and function by glucocorticoids in mice
    B Valtat
    INSERM UMR S 872, Centre de Recherche des Cordeliers, 15 rue de l Ecole de Medecine, 75006 Paris, France
    Diabetologia 54:350-9. 2011
    ..Our aim was to further dissect the role of glucocorticoids on beta cell development and function in mice...
  43. pmc Glucose stimulation of hypothalamic MCH neurons involves K(ATP) channels, is modulated by UCP2, and regulates peripheral glucose homeostasis
    Dong Kong
    Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA
    Cell Metab 12:545-52. 2010
    ..Combined, the glucose-excited neurons are likely to play key, previously unexpected roles in regulating blood glucose...
  44. pmc ATP modulates interaction of syntaxin-1A with sulfonylurea receptor 1 to regulate pancreatic beta-cell KATP channels
    Youhou Kang
    Department of Medicine, University of Toronto and University Health Network, Toronto, Ontario M5S 1A8, Canada
    J Biol Chem 286:5876-83. 2011
    ..endogenous membrane-bound SNARE protein syntaxin-1A (Syn-1A), which binds both nucleotide-binding folds of sulfonylurea receptor (SUR)1 and 2A, causing inhibition of K(ATP) channel activity in pancreatic islet β-cells and cardiac ..
  45. pmc Conserved intramolecular disulfide bond is critical to trafficking and fate of ATP-binding cassette (ABC) transporters ABCB6 and sulfonylurea receptor 1 (SUR1)/ABCC8
    Yu Fukuda
    Department of Pharmaceutical Sciences, St Jude Children s Research Hospital, Memphis, Tennessee 38105, USA
    J Biol Chem 286:8481-92. 2011
    ..This analysis led to the discovery of a disease-causing mutation in the sulfonylurea receptor 1 (SUR1)/ABCC8 from a patient with hyperinsulinemic hypoglycemia...
  46. pmc Glucose transporters and ATP-gated K+ (KATP) metabolic sensors are present in type 1 taste receptor 3 (T1r3)-expressing taste cells
    Karen K Yee
    Monell Chemical Senses Center, Philadelphia, PA 19104 3308, USA
    Proc Natl Acad Sci U S A 108:5431-6. 2011
    ..sodium-glucose cotransporter (SGLT1), and two components of the ATP-gated K(+) (K(ATP)) metabolic sensor [sulfonylurea receptor (SUR) 1 and potassium inwardly rectifying channel (Kir) 6.1] were expressed selectively in taste cells...
  47. pmc The thyroid hormone-inactivating type III deiodinase is expressed in mouse and human beta-cells and its targeted inactivation impairs insulin secretion
    Mayrin C Medina
    Division of Endocrinology, Diabetes, and Metabolism, University of Miami Miller School of Medicine, Miami, Florida 33136, USA
    Endocrinology 152:3717-27. 2011
    ..An analogous role is likely in humans, given the similar D3 expression pattern...
  48. ncbi Identification of 18 mouse ABC genes and characterization of the ABC superfamily in Mus musculus
    L M Schriml
    Human Genetics Section, Laboratory of Genomic Diversity, National Cancer Institute Frederick Cancer Research and Development Center, Frederick, MD 21702, USA
    Genomics 64:24-31. 2000
    ..The mouse ABC genes could be grouped into the seven previously described human ABC subfamilies. Three mouse ABC genes mapped to regions implicated in cholesterol gallstone susceptibility...
  49. ncbi Expression of sulphonylurea receptor protein in mouse kidney
    A H Beesley
    Laboratory for Membrane Protein Function, Department of Biomedical Science, The University of Sheffield, Western Bank, Sheffield S10 2TN, UK
    Pflugers Arch 438:1-7. 1999
    The sulphonylurea receptor (SUR) is the site of action for sulphonylurea derivatives such as glibenclamide, which are widely used as oral hypoglycaemic agents...
  50. pmc Alternative sulfonylurea receptor expression defines metabolic sensitivity of K-ATP channels in dopaminergic midbrain neurons
    B Liss
    Institute for Neural Signal Transduction, Centre for Molecular Neurobiology, Martinistrasse 52, 20246 Hamburg, Germany
    EMBO J 18:833-46. 1999
    ..Several isoforms of the K-ATP channel subunits, the sulfonylurea receptor (SUR) and inwardly rectifying K channel (Kir6...
  51. ncbi Long chain coenzyme A esters activate the pore-forming subunit (Kir6. 2) of the ATP-regulated potassium channel
    R Branstrom
    Rolf Luft Center for Diabetes Research, Department of Molecular Medicine, Karolinska Institute, S 171 76 Stockholm, Sweden
    J Biol Chem 273:31395-400. 1998
    ..2 and the sulfonylurea receptor type 1 (SUR1). Both subunits are required for functional KATP channels because expression of Kir6...
  52. ncbi Developmental and tissue-specific sulfonylurea receptor gene expression
    C Hernandez-Sanchez
    Section on Molecular and Cellular Physiology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892 1770, USA
    Endocrinology 138:705-11. 1997
    We have studied the developmental regulation of mouse sulfonylurea receptor (SUR) gene expression throughout several embryonic stages as well as in the adult mouse...
  53. ncbi Molecular mapping of the tubby (tub) mutation on mouse chromosome 7
    W K Chung
    The Rockefeller University, New York, New York 10021, USA
    Genomics 32:210-7. 1996
    ..The gene sulfonylurea receptor was eliminated as a candidate gene for tubby on the basis of its map position, approximately 3.1 +/- 3...
  54. ncbi Cloning of the beta cell high-affinity sulfonylurea receptor: a regulator of insulin secretion
    L Aguilar-Bryan
    Department of Cell Biology, Baylor College of Medicine, Houston, TX 77030, USA
    Science 268:423-6. 1995
    ..These drugs interact with the sulfonylurea receptor of pancreatic beta cells and inhibit the conductance of adenosine triphosphate (ATP)-dependent potassium (..
  55. ncbi A signaling role of glutamine in insulin secretion
    Changhong Li
    Division of Endocrinology, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 279:13393-401. 2004
    ..the effects of amino acids on insulin secretion and intracellular calcium in islets from normal and sulfonylurea receptor 1 knockout (SUR1-/-) mice...
  56. ncbi Cholinergic regulation of fuel-induced hormone secretion and respiration of SUR1-/- mouse islets
    Nicolai M Doliba
    Dept of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Am J Physiol Endocrinol Metab 291:E525-35. 2006
    ..restore defective glucose-stimulated insulin release in pancreatic islets lacking sulfonylurea type 1 receptors (SUR1(-/-)) (Doliba NM, Qin W, Vatamaniuk MZ, Li C, Zelent D, Najafi H, Buettger CW, Collins HW, Carr RD, Magnuson MA, ..
  57. ncbi Fine mapping of Dyscalc1, the major genetic determinant of dystrophic cardiac calcification in mice
    Susanne Korff
    Division of Cardiology, Department of Medicine III, University of Heidelberg, Heidelberg, Germany
    Physiol Genomics 25:387-92. 2006
    ..Mutations in the orthologous human ABCC6 gene cause pseudoxanthoma elasticum, or Gronblad-Strandberg syndrome, an elastic tissue disorder with cardiovascular calcifications...
  58. ncbi Intracellular ATP-sensitive K+ channels in mouse pancreatic beta cells: against a role in organelle cation homeostasis
    A Varadi
    Henry Wellcome Laboratories for Integrated Cell Signalling and Department of Biochemistry, School of Medical Sciences, University of Bristol, University Walk, Bristol, BS8 1TD, UK
    Diabetologia 49:1567-77. 2006
    ..The aim of this study was to explore these possibilities and to test the hypothesis that vesicle-resident channels play a role in the control of organellar Ca(2+) concentration or pH...
  59. ncbi Crosstalk between membrane potential and cytosolic Ca2+ concentration in beta cells from Sur1-/- mice
    D Haspel
    Department of Pharmacology, Institute of Pharmacy, University of Tubingen, Auf der Morgenstelle 8, 72076 Tubingen, Germany
    Diabetologia 48:913-21. 2005
    Islets or beta cells from Sur1(-/-) mice were used to determine whether changes in plasma membrane potential (V(m)) remain coupled to changes in cytosolic Ca(2+) ([Ca(2+)](i)) in the absence of K(ATP) channels and thus provide a ..
  60. ncbi Similarities of K+ATP channel expression and Ca2+ changes in pancreatic beta cells and hypothalamic neurons
    Paul E Squires
    Molecular Physiology, Department of Biological Sciences, University of Warwick, Coventry, United Kingdom
    Pancreas 30:227-32. 2005
    ....
  61. ncbi Resveratrol binds to the sulfonylurea receptor (SUR) and induces apoptosis in a SUR subtype-specific manner
    Annette Hambrock
    Department of Pharmacology and Toxicology, Medical Faculty, University of Tubingen, Wilhelmstrasse 56, D 72074 Tubingen, Germany
    J Biol Chem 282:3347-56. 2007
    ..Electrophysiological measurements revealed that resveratrol is a blocker of pancreatic SUR1/K(IR)6.2 K(ATP) channels. We further demonstrate that, like glibenclamide, resveratrol induces enhanced apoptosis...
  62. ncbi PED/PEA-15 regulates glucose-induced insulin secretion by restraining potassium channel expression in pancreatic beta-cells
    Claudia Miele
    Department of Cellular and Molecular Biology and Pathology, Federico II University of Naples, Via Sergio Pansini 5, Naples 80131, Italy
    Diabetes 56:622-33. 2007
    ..Islets from the beta-tg also exhibited little response to glucose. mRNAs encoding the Sur1 and Kir6.2 potassium channel subunits and their upstream regulator Foxa2 were specifically reduced in these islets...
  63. ncbi Docking and fusion of insulin secretory granules in SUR1 knock out mouse beta-cells observed by total internal reflection fluorescence microscopy
    Toshiteru Kikuta
    Department of Biochemistry, Kyorin University School of Medicine, 6 20 2 Shinkawa, Mitaka, Tokyo 181 8611, Japan
    FEBS Lett 579:1602-6. 2005
    To explore how the sulfonylurea receptor (SUR1) is involved in docking and fusion of insulin granules, dynamic motion of single insulin secretory granules near the plasma membrane was examined in SUR1 knock-out (Sur1KO) beta-cells by ..
  64. ncbi Arrhythmia susceptibility and premature death in transgenic mice overexpressing both SUR1 and Kir6.2[DeltaN30,K185Q] in the heart
    Thomas P Flagg
    Department of Cell Biology and Physiology, Washington University School of Medicine, 660 S Euclid Avenue, St Louis, MO 63110, USA
    Am J Physiol Heart Circ Physiol 293:H836-45. 2007
    ..2 mutant (Kir6.2[DeltaN30,K185Q]) or the accessory sulfonylurea receptor (SUR)2A (FLAG-SUR2A) or SUR1 (FLAG-SUR1) subunits of the K(ATP) channel, under transcriptional control of ..
  65. ncbi Morphological localisation of sulfonylurea receptor 1 in endocrine cells of human, mouse and rat pancreas
    Y Guiot
    Department of Pathology, Faculty of Medicine, University of Louvain, B 1200, Brussels, Belgium
    Diabetologia 50:1889-99. 2007
    b>Sulfonylurea receptor 1 (SUR1) is the regulatory subunit of ATP-sensitive K channels in beta cells...
  66. ncbi Expression of ATP-sensitive K+ channel subunits during perinatal maturation in the mouse heart
    Alison Morrissey
    Department of Pediatrics, NYU School of Medicine, New York, New York 10016, USA
    Pediatr Res 58:185-92. 2005
    ..2 and SUR2A subunits, but the expression of other K(ATP) channel subunits (including SUR1, SUR2B, and Kir6.1) is poorly defined...
  67. doi Role of sulfonylurea receptor type 1 subunits of ATP-sensitive potassium channels in myocardial ischemia/reperfusion injury
    John W Elrod
    Department of Medicine and Pathology, Albert Einstein College of Medicine, New York, NY, USA
    Circulation 117:1405-13. 2008
    ..2 (inward-rectifier potassium channel 6.2) and SUR2A (sulfonylurea receptor type 2A) subunits; however, the evidence is strong that SUR1 (sulfonylurea receptor type 1) subunits are ..
  68. pmc Elimination of KATP channels in mouse islets results in elevated [U-13C]glucose metabolism, glutaminolysis, and pyruvate cycling but a decreased gamma-aminobutyric acid shunt
    Changhong Li
    Division of Endocrinology, The Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 283:17238-49. 2008
    ..cells are hyper-responsive to amino acids but have decreased glucose sensitivity after deletion of the sulfonylurea receptor 1 (SUR1) both in man and mouse...
  69. ncbi Restitution of defective glucose-stimulated insulin release of sulfonylurea type 1 receptor knockout mice by acetylcholine
    Nicolai M Doliba
    Diabetes Research Center, Department of Biochemistry and Biophysics, Univ of Pennsylvania, Philadelphia, PA 19104 6015, USA
    Am J Physiol Endocrinol Metab 286:E834-43. 2004
    ..It is therefore surprising that mice lacking the sulfonylurea type 1 receptor (SUR1-/-) in beta-cells remain euglycemic even though the knockout is expected to cause hypoglycemia...
  70. pmc Disruption of sarcolemmal ATP-sensitive potassium channel activity impairs the cardiac response to systolic overload
    Xinli Hu
    Cardiovascular Division, Department of Medicine, Universityof Minnesota Medical School, Minneapolis, MN 55455, USA
    Circ Res 103:1009-17. 2008
    ..ventricular response to stress using transgenic mouse strains with cardiac-specific disruption of K(ATP) activity (SUR1-tg mice) or Kir6.2 gene deficiency (Kir6.2 KO). Both SUR1-tg and Kir6...
  71. pmc Fasting-induced suppression of LH secretion does not require activation of ATP-sensitive potassium channels
    Wenyu Huang
    Department of Neurobiology and Physiology, Northwestern University, Evanston, Illinois, USA
    Am J Physiol Endocrinol Metab 295:E1439-46. 2008
    ..In sulfonylurea (SUR)1-null mutant (SUR1(-/-)) mice, which are deficient in K(ATP) channels, and their wild-type (WT) littermates, a 48-h fast was found to ..
  72. ncbi Epinephrine-induced hyperpolarization of islet cells without KATP channels
    Andrea Sieg
    Institut fur Neurophysiologie, Universitat zu Koln, Cologne, Germany
    Am J Physiol Endocrinol Metab 286:E463-71. 2004
    ..physiological inhibitor of insulin secretion, on the membrane potential of pancreatic islet cells from sulfonylurea receptor-1 (ABCC8)-null mice (Sur1KO), which lack functional ATP-sensitive K+ (KATP) channels...
  73. doi 17beta-Estradiol modulates apoptosis in pancreatic beta-cells by specific involvement of the sulfonylurea receptor (SUR) isoform SUR1
    Stefanie Ackermann
    Department of Pharmacology and Toxicology, Medical Faculty, University of Tubingen, Wilhelmstrasse 56, D 72074 Tubingen, Germany
    J Biol Chem 284:4905-13. 2009
    ..shown that the "phytoestrogen" resveratrol can induce beta-cell apoptosis dependent on the expression of sulfonylurea receptor (SUR) 1, the regulatory subunit of pancreatic ATP-sensitive K(+) channels...
  74. doi Impact of Sur1 gene inactivation on the morphology of mouse pancreatic endocrine tissue
    Ihsane Marhfour
    Department of Pathology, Faculty of Medicine, Universite Catholique de Louvain, Brussels, Belgium
    Cell Tissue Res 335:505-15. 2009
    In congenital hyperinsulinism of infancy (CHI), the loss of K-ATP channels (composed of Kir6.2 and SUR1 subunits) in beta cells induces permanent insulin secretion and severe hypoglycaemia...
  75. pmc SUR1 regulates PKA-independent cAMP-induced granule priming in mouse pancreatic B-cells
    Lena Eliasson
    Department of Molecular and Cellular Physiology, Institute of Physiological Sciences, SE 221 84 Lund, Sweden
    J Gen Physiol 121:181-97. 2003
    ..Surprisingly, B-cells in islets isolated from SUR1-deficient mice (SUR1(-/-) mice) lacked the PKA-independent component of exocytosis...
  76. ncbi Modulation of the trafficking efficiency and functional properties of ATP-sensitive potassium channels through a single amino acid in the sulfonylurea receptor
    Etienne A Cartier
    Center for Research on Occupational and Environmental Toxicology, Oregon Health and Science University, Portland, Oregon 97201, USA
    J Biol Chem 278:7081-90. 2003
    Mutations in the sulfonylurea receptor 1 (SUR1), a subunit of ATP-sensitive potassium (K(ATP)) channels, cause familial hyperinsulinism...

Research Grants7

  1. REGULATION OF ADIPOCYTE PROTEIN SYNTHESIS BY AMINO ACIDS
    Christopher Lynch; Fiscal Year: 2007
    ..The proposed studies will elucidate how leucine regulates mTOR and the roles of leucine stimulation of mTOR in adipose tissue physiology and obesity. ..
  2. Role of Leucine Metabolism in Leucine Signaling
    CHRISTOPHER JOHN LYNCH; Fiscal Year: 2010
    ..The implication of our ongoing research is that blocking the first step in BCAA metabolism with a drug, or to a less extent increasing dietary BCAAs, will promote weight loss. ..
  3. PATHOPHYSIOLOGY OF NICOTINE IN CEREBRAL BLOOD VESSELS
    J Simard; Fiscal Year: 2004
    ....
  4. CEREBROVASCULAR ION CHANNELS IN HYPERTENSION
    J Marc Simard; Fiscal Year: 2007
    ..abstract_text> ..
  5. Reactive astrocytes from gliotic capsule
    J Marc Simard; Fiscal Year: 2007
    ..unreadable] [unreadable]..
  6. Role of GLP-1 in disorders of carbohydrate metabolism in children
    DIVA DE LEON; Fiscal Year: 2008
    ....
  7. Effect of exendin-(9-39) on glucose metabolism in subjects with hyperinsulinism
    DIVA DE LEON; Fiscal Year: 2008
    ..Mutations in five genes have been associated with CHI: the sulfonylurea receptor (SUR-1), an inward rectifying potassium channel (Kir6...