Current understanding of KATP channels in neonatal diseases: Focus on insulin secretion disorders

Acta Pharmacologica Sinica

Volumn 32, Issue 6, 2011, Pages 765-780

  Quan, Yi ^a   Barszczyk, Andrew ^a   Feng, Zhong Ping ^a   Sun, Hong Shuo ^a,b  

^a UNIVERSITY OF TORONTO   (Canada)

^b UNIVERSITY OF TORONTO   (Canada)

Author keywords

ATP sensitive potassium (KATP) channels; channelopathy; congenital hyperinsulinism; diazoxide; glucose homeostasis; neonatal diabetes; sulfonylureas

Indexed keywords

ADENOSINE TRIPHOSPHATE SENSITIVE POTASSIUM CHANNEL; GLUCOSE;

CONFERENCE PAPER; DIABETES MELLITUS; GENE MUTATION; GLUCOSE BLOOD LEVEL; GLUCOSE HOMEOSTASIS; HUMAN; INSULIN RELEASE; NEWBORN DISEASE; NONHUMAN; PANCREAS ISLET BETA CELL; PATHOPHYSIOLOGY; PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY;

ANIMALS; DIABETES MELLITUS; GLUCOSE; HOMEOSTASIS; HUMANS; INFANT, NEWBORN; INSULIN; KATP CHANNELS; PERSISTENT HYPERINSULINEMIA HYPOGLYCEMIA OF INFANCY;

EID: 79958118313     PISSN: 16714083     EISSN: 17457254     Source Type: Journal    
DOI: 10.1038/aps.2011.57     Document Type: Conference Paper

References (224)

1. ATP channel complex. J Gen Physiol 1997;110:655-64. (Pubitemid 27524606)
2. Hibino H, Inanobe A, Furutani K, Murakami S, Findlay I, Kurachi Y. Inwardly rectifying potassium channels: their structure, function, and physiological roles. Physiol Rev 2010;90:291-366.
3. ATP channels as molecular sensors of cellular metabolism. Nature 2006;440:470-6.
4. + channels in cardiac muscle. Nature 1983;305:147-8. (Pubitemid 14235671)
5. Ashcroft FM, Harrison DE, Ashcroft SJ. Glucose induces closure of single potassium channels in isolated rat pancreatic beta-cells. Nature 1984;312:446-8. (Pubitemid 15217837)
6. + channels in pancreatic beta-cells. Nature 1984;311:271-3. (Pubitemid 14071146)
7. Spruce AE, Standen NB, Stanfield PR. Studies of the unitary properties of adenosine-5′-triphosphate-regulated potassium channels of frog skeletal muscle. J Physiol 1987;382:213-36. (Pubitemid 17226144)
8. Spruce AE, Standen NB, Stanfield PR. Voltage-dependent ATP-sensitive potassium channels of skeletal muscle membrane. Nature 1985;316:736-8. (Pubitemid 16239351)
9. + channels in arterial smooth muscle. Science 1989;245:177-80. (Pubitemid 19193933)
10. Gabrielsson BG, Karlsson AC, Lonn M, Olofsson LE, Johansson JM, Torgerson JS, et al. Molecular characterization of a local sulfonylurea system in human adipose tissue. Mol Cell Biochem 2004;258:65-71. (Pubitemid 38268201)
11. Zawar C, Plant TD, Schirra C, Konnerth A, Neumcke B. Cell-type specific expression of ATP-sensitive potassium channels in the rat hippo campus. J Physiol 1999;514:327-41. (Pubitemid 29055963)
12. Ashford ML, Sturgess NC, Trout NJ, Gardner NJ, Hales CN. Adenosine-5′-triphosphate-sensitive ion channels in neonatal rat cultured central neurones. Pfugers Arch 1988;412:297-304.
13. + channels. Pflugers Arch 1990;415:479-83. (Pubitemid 20040708)
14. + channels. Br J Pharmacol 1990;101:531-40. (Pubitemid 20357171)
15. Hattersley AT, Ashcroft FM. Activating mutations in Kir6.2 and neonatal diabetes: new clinical syndromes, new scientific insights, and new therapy. Diabetes 2005;54:2503-13. (Pubitemid 41233571)
16. Nishida M, Cadene M, Chait BT, MacKinnon R. Crystal structure of a Kir3.1-prokaryotic Kir channel chimera. EMBO J 2007;26:4005-15. (Pubitemid 47367496)
17. Aguilar-Bryan L, Nichols CG, Wechsler SW, Clement JP, Boyd AE III, Gonzalez G, et al. Cloning of the beta cell high-affinity sulfonylurea receptor: a regulator of insulin secretion. Science 1995;268:423-6.
18. Babenko AP, Bryan J. SUR-dependent modulation of KAT P channels by an N-terminal KIR6.2 peptide. Defining intersubunit gating interactions. J Biol Chem 2002;277:43997-4004. (Pubitemid 36157825)
19. + channels. Neuron 1996;16:1011-7. (Pubitemid 26171205)
20. + channel. J Biol Chem 1996;271:24321-4. (Pubitemid 26333158)
21. Proks P, Capener CE, Jones P, Ashcroft FM. Mutations within the P-loop of Kir6.2 modulate the intraburst kinetics of the ATP-sensitive potassium channel. J Gen Physiol 2001;118:341-53. (Pubitemid 32979999)
22. Jiang Y, Lee A, Chen J, Cadene M, Chait BT, MacKinnon R. The open pore conformation of potassium channels. Nature 2002;417:523-6. (Pubitemid 34595913)
23. Enkvetchakul D, Nichols CG. Gating mechanism of KAT P channels: function fits form. J Gen Physiol 2003;122:471-80. (Pubitemid 37420843)
24. Sackin H, Nanazashvili M, Palmer LG, Krambis M, Walters DE. Structural locus of the pH gate in the Kir1.1 inward rectifier channel. Biophys J 2005;88:2597-606. (Pubitemid 40976128)
25. ATP channel inhibition by ATP. Biophys J 2004;86:2101-12. (Pubitemid 38524400)
26. Enkvetchakul D, Loussouarn G, Makhina E, Shyng SL, Nichols CG. The kinetic and physical basis of KAT P channel gating: toward a unified molecular understanding. Biophys J 2000;78:2334-48. (Pubitemid 30313793)
27. Drain P, Li L, Wang J. KAT P channel inhibition by ATP requires distinct functional domains of the cytoplasmic C terminus of the pore-forming subunit. Proc Natl Acad Sci U S A 1998;95:13953-8.
28. ATP channels. Neuron 1999;22:537-48. (Pubitemid 29159852)
29. Hosy E, Dupuis JP, Vivaudou M. Impact of disease-causing SUR1 mutations on the KAT P channel subunit interface probed with a rhoda-mine protection assay. J Biol Chem 2010;285:3084-91.
30. ATP channel expression and activity by the SUR1 nucleotide binding fold 1. Channels (Austin) 2007;1:315-23.
31. ATP channels. J Biol Chem 2002;277:17139-46. (Pubitemid 34967746)
32. ATP channel trafficking via AMP-activated protein kinase in pancreatic beta-cells. Diabetes 2009;58:2813-9.
33. ATP channel Kir6.2 subunit. EMBO J 2005;24:229-39. (Pubitemid 40216128)
34. Dong K, Tang LQ, MacGregor GG, Leng Q, Hebert SC. Novel nucleotide-binding sites in ATP-sensitive potassium channels formed at gating interfaces. EMBO J 2005;24:1318-29. (Pubitemid 40593053)
35. Markworth E, Schwanstecher C, Schwanstecher M. ATP4-mediates closure of pancreatic beta-cell ATP-sensitive potassium channels by interaction with 1 of 4 identical sites. Diabetes 2000;49:1413-8.
36. ATP channels. J Gen Physiol 2002;120:437-46. (Pubitemid 35014739)
37. + channel Kir6.2. J Physiol 2003;552:23-34. (Pubitemid 37265465)
38. ATP channel inhibition by ATP. Biophys J 2000;79:841-52. (Pubitemid 30626178)
39. ATP channel by ATP. J Physiol 1999;514:19-25. (Pubitemid 29033885)
40. Reimann F, Ryder TJ, Tucker SJ, Ashcroft FM. The role of lysine 185 in the Kir6.2 subunit of the ATP-sensitive channel in channel inhibition by ATP. J Physiol 1999;520:661-9. (Pubitemid 29533026)
41. + channel Kir6.2 revealed by fluorescence resonance energy transfer. Proc Natl Acad Sci U S A 2004;101:76-81. (Pubitemid 38084206)
42. ATP channel inhibition by ATP. EMBO J 1998;17:3290-6. (Pubitemid 28279503)
43. Trapp S, Haider S, Jones P, Sansom MS, Ashcroft FM. Identification of residues contributing to the ATP binding site of Kir6.2. EMBO J 2003;22:2903-12. (Pubitemid 36758612)
44. Gloyn AL, Pearson ER, Antcliff JF, Proks P, Bruining GJ, Slingerland AS, 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. (Pubitemid 38917250)
45. Proks P, Girard C, Haider S, Gloyn AL, Hattersley AT, Sansom MS, et al. A gating mutation at the internal mouth of the Kir6.2 pore is associated with DEND syndrome. EMBO Rep 2005;6:470-5. (Pubitemid 40767081)
46. 2+ ions. J Physiol 1989;416:349-67. (Pubitemid 19223219)
47. Campbell JD, Proks P, Lippiat JD, Sansom MS, Ashcroft FM. Identifi-ca tion of a functionally important negatively charged residue within the second catalytic site of the SUR1 nucleotide-binding domains. Diabetes 2004;53: S123-7. (Pubitemid 39564533)
48. Masia R, Nichols CG. Functional clustering of mutations in the dimer interface of the nucleotide binding folds of the sulfonylurea receptor. J Biol Chem 2008;283:30322-9.
49. Vergani P, Lockless SW, Nairn AC, Gadsby DC. CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains. Nature 2005;433:876-80. (Pubitemid 40314897)
50. + channels. Mol Pharmacol 2004;65:1198-207. (Pubitemid 38543490)
51. Dunne MJ, Cosgrove KE, Shepherd RM, Aynsley-Green A, Lindley KJ. Hyperinsulinism in infancy: from basic science to clinical disease. Physiol Rev 2004;84:239-75. (Pubitemid 38049881)
52. Nichols CG, Shyng SL, Nestorowicz A, Glaser B, Clement JP, Gonzalez G, et al. Adenosine diphosphate as an intracellular regulator of insulin secretion. Science 1996;272:1785-7. (Pubitemid 26256397)
53. Sharma N, Crane A, Gonzalez G, Bryan J, Aguilar-Bryan L. Familial hyper insulinism and pancreatic beta-cell ATP-sensitive potassium channels. Kidney Int 2000;57:803-8. (Pubitemid 30434769)
54. Otonkoski T, Ammala C, Huopio H, Cote GJ, Chapman J, Cosgrove K, et al. A point mutation inactivating the sulfonylurea receptor causes the severe form of persistent hyperinsulinemic hypoglycemia of infancy in Finland. Diabetes 1999;48:408-15. (Pubitemid 29061118)
55. ATP pores define a novel gatekeeper. J Biol Chem 2003;278:41577-80. (Pubitemid 37310410)
56. ATP channels. J Physiol 2006;576:379-89. (Pubitemid 44490132)
57. Hosy E, Derand R, Revilloud J, Vivaudou M. Remodelling of the SUR-Kir6.2 interface of the KAT P channel upon ATP binding revealed by the conformational blocker rhodamine 123. J Physiol 2007;582:27-39. (Pubitemid 46993559)
58. ATP channel. J Biol Chem 2004;279:4234-40. (Pubitemid 38199010)
59. ATP channels by its actions on the sulfonylurea receptor 1 nucleotide-binding folds-1 and-2. J Biol Chem 2004;279:53259-65. (Pubitemid 40051829)
60. Kang Y, Leung YM, Manning-Fox JE, Xia F, Xie H, Sheu L, et al. Syntaxin-1A inhibits cardiac KAT P channels by its actions on nucleotide binding folds 1 and 2 of sulfonylurea receptor 2A. J Biol Chem 2004;279:47125-31. (Pubitemid 39518369)
61. ATP channels. J Biol Chem 2011;286:5876-83.
62. Chen PC, Bruederle CE, Gaisano HY, Shyng SL. Syntaxin 1A regulates surface expression of {beta}-cell ATP-sensitive potassium channels. Am J Physiol Cell Physiol 2011;300: C506-16.
63. Ng B, Kang Y, Xie H, Sun H, Gaisano HY. Syntaxin-1A inhibition of P-1075, cromakalim, and diazoxide actions on mouse cardiac ATP-sensitive potassium channel. Cardiovasc Res 2008;80:365-74.
64. + channel opener can be modulated by syntaxin-1A acting on sulfonylurea receptor 1. Diabetes 2007;56:2124-34. (Pubitemid 47195828)
65. ATP channels. Science 1998;282:1141-4. (Pubitemid 28516246)
66. Shyng SL, Cukras CA, Harwood J, Nichols CG. Structural determinants of PIP (2) regulation of inward rectifier KAT P channels. J Gen Physiol 2000;116:599-608.
67. Shyng SL, Barbieri A, Gumusboga A, Cukras C, Pike L, Davis JN, et al. Modulation of nucleotide sensitivity of ATP-sensitive potassium channels by phosphatidylinositol-4-phosphate 5-kinase. Proc Natl Acad Sci U S A 2000;97:937-41. (Pubitemid 30070413)
68. + channel. Circ Res 2004;94:1359-66. (Pubitemid 38724482)
69. Shi Y, Wu Z, Cui N, Shi W, Yang Y, Zhang X, et al. PKA phosphorylation of SUR2B subunit underscores vascular KAT P channel activation by beta-adrenergic receptors. Am J Physiol Regul Integr Comp Physiol 2007;293: R1205-14. (Pubitemid 47358942)
70. Van Wagoner DR. Mechanosensitive gating of atrial ATP-sensitive potassium channels. Circ Res 1993;72:973-83. (Pubitemid 23105503)
71. Light PE, Bladen C, Winkfein RJ, Walsh MP, French RJ. Molecular basis of protein kinase C-induced activation of ATP-sensitive potassium channels. Proc Natl Acad Sci U S A 2000;97:9058-63. (Pubitemid 30626673)
72. Ross R, Wang PY, Chari M, Lam CK, Caspi L, Ono H, et al. Hypotha-lamic protein kinase C regulates glucose production. Diabetes 2008;57:2061-5.
73. Hu K, Huang CS, Jan YN, Jan LY. ATP-sensitive potassium channel traffic regulation by adenosine and protein kinase C. Neuron 2003;38:417-32. (Pubitemid 36579144)
74. + channel? Studies of contractile function after simulated ischemia in an atrial in vitro model. Circ Res 1995;77:1030-5.
75. + current in human and rabbit ventricular myocytes. Circ Res 1996;78:492-8. (Pubitemid 26072549)
76. + channels. A possible mechanistic link to ischemic preconditioning. Circ Res 1996;79:399-406. (Pubitemid 26276900)
77. Zhuang JG, Zhang Y, Zhou ZN. Hypoxic preconditioning upregulates KAT P channels through activation of protein kinase C in rat ventricular myocytes. Acta Pharmacol Sin 2000;21:845-9.
78. + channels in excised membrane patches is mediated by PKC. Am J Physiol 1999;276: H488-95. (Pubitemid 29100616)
79. Nathan DM, Buse JB, Davidson MB, Ferrannini E, Holman RR, Sherwin R, et al. Medical management of hyperglycemia in type 2 diabetes: a consensus algorithm for the initiation and adjustment of therapy: a consensus statement of the American Diabetes Association and the European Association for the Study of Diabetes. Diabetes Care 2009;32:193-203.
80. Greeley SA, Tucker SE, Naylor RN, Bell GI, Philipson LH. Neonatal diabetes mellitus: a model for personalized medicine. Trends Endocrinol Metab 2010;21:464-72.
81. Gribble FM, Reimann F. Sulphonylurea action revisited: the post-cloning era. Diabetologia 2003;46:875-91. (Pubitemid 36961311)
82. ATP channel. Diabetes 1999;48:1341-7. (Pubitemid 29241036)
83. ATP channel. FEBS Lett 2001;499:154-60. (Pubitemid 32539340)
84. + channel currents expressed in Xenopus oocytes: a reinterpretation. J Physiol 1997;504:35-45. (Pubitemid 27464270)
85. ATP channel. Biochem Biophys Res Commun 1999;257:766-70. (Pubitemid 29301515)
86. Arnoux JB, de LP, Ribeiro MJ, Hussain K, Blankenstein O, Mohnike K, et al. Congenital hyperinsulinism. Early Hum Dev 2010;86:287-94.
87. Kapoor RR, Flanagan SE, James C, Shield J, Ellard S, Hussain K. Hyperinsulinaemic hypoglycaemia. Arch Dis Child 2009;94:450-7.
88. ATP channel openers. Trends Pharmacol Sci 2000;21:439-45.
89. + channels in the cardiovascular system. J Cardiovasc Electrophysiol 1997;8:1431-46. (Pubitemid 28013656)
90. + channel-opening drugs. Am J Physiol 1995;269: C525-45.
91. Quayle JM, Nelson MT, Standen NB. ATP-sensitive and inwardly rectifying potassium channels in smooth muscle. Physiol Rev 1997;77:1165-232. (Pubitemid 27459582)
92. Moreau C, Jacquet H, Prost AL, D'hahan N, Vivaudou M. The molecular basis of the specificity of action of KAT P channel openers. EMBO J 2000;19:6644-51. (Pubitemid 32011657)
93. ATP channel openers. Mol Pharmacol 2005;67:1026-33. (Pubitemid 40410405)
94. Moreau C, Prost AL, Derand R, Vivaudou M. SUR, ABC proteins targeted by KAT P channel openers. J Mol Cell Cardiol 2005;38:951-63. (Pubitemid 40725943)
95. ATP channel-deficient mice. Proc Natl Acad Sci U S A 1998;95:10402-6.
96. + channels as metabolic sensors: studies of Kir6.x null mice. Diabetes 2004;53: S176-80. (Pubitemid 39564541)
97. Franklin I, Gromada J, Gjinovci A, Theander S, Wollheim CB. Beta-cell secretory products activate alpha-cell ATP-dependent potassium channels to inhibit glucagon release. Diabetes 2005;54:1808-15. (Pubitemid 40770771)
98. MacDonald PE, De Marinis YZ, Ramracheya R, Salehi A, Ma X, Johnson PR, et al. A KAT P channel-dependent pathway within alpha cells regulates glucagon release from both rodent and human islets of Langerhans. PLoS Biol 2007;5: e143.
99. Gromada J, Franklin I, Wollheim CB. Alpha-cells of the endocrine pancreas: 35 years of research but the enigma remains. Endocr Rev 2007;28:84-116. (Pubitemid 46220851)
100. Oomura Y, Ono T, Ooyama H, Wayner MJ. Glucose and osmosensitive neurones of the rat hypothalamus. Nature 1969;222:282-4.
101. Taborsky GJ Jr, Ahren B, Havel PJ. Autonomic mediation of glucagon secretion during hypoglycemia: implications for impaired alpha-cell responses in type 1 diabetes. Diabetes 1998;47:995-1005. (Pubitemid 28294539)
102. + channels in the hypothalamus are essential for the maintenance of glucose homeostasis. Nat Neurosci 2001;4:507-12. (Pubitemid 32397188)
103. ATP channels control hepatic glucose production. Nature 2005;434:1026-31. (Pubitemid 40586051)
104. Konner AC, Janoschek R, Plum L, Jordan SD, Rother E, Ma X, et al. Insulin action in AgRP-expressing neurons is required for suppression of hepatic glucose production. Cell Metab 2007;5:438-49. (Pubitemid 46825497)
105. Parton LE, Ye CP, Coppari R, Enriori PJ, Choi B, Zhang CY, et al. Glucose sensing by POMC neurons regulates glucose homeostasis and is impaired in obesity. Nature 2007;449:228-32. (Pubitemid 47402365)
106. + currents. Mol Endocrinol 2007;21:765-73.
107. ATP channels enhances insulin-stimulated glucose uptake in skeletal muscle. Proc Natl Acad Sci U S A 2001;98:11760-4. (Pubitemid 32928802)
108. Miki T, Minami K, Zhang L, Morita M, Gonoi T, Shiuchi T, et al. ATP-sensi tive potassium channels participate in glucose uptake in skeletal muscle and adipose tissue. Am J Physiol Endocrinol Metab 2002;283: E1178-84. (Pubitemid 35340624)
109. Araki E, Lipes MA, Patti ME, Bruning JC, Haag B3, Johnson RS, et al. Alternative pathway of insulin signalling in mice with targeted disruption of the IRS-1 gene. Nature 1994;372:186-90. (Pubitemid 24347839)
110. + channel-mediated glucose uptake is independent of IRS-1/phosphatidylinositol 3-kinase signaling. Am J Physiol Endocrinol Metab 2003;285: E1289-96. (Pubitemid 37464380)
111. Tamemoto H, Kadowaki T, Tobe K, Yagi T, Sakura H, Hayakawa T, et al. Insulin resistance and growth retardation in mice lacking insulin receptor substrate-1. Nature 1994;372:182-6. (Pubitemid 24347838)
112. Mu J, Brozinick JT Jr, Valladares O, Bucan M, Birnbaum MJ. A role for AMP-activated protein kinase in contraction-and hypoxia-regulated glucose transport in skeletal muscle. Mol Cell 2001;7:1085-94. (Pubitemid 32525754)
113. Musi N, Fujii N, Hirshman MF, Ekberg I, Froberg S, Ljungqvist O, et al. AMP-activated protein kinase (AMPK) is activated in muscle of subjects with type 2 diabetes during exercise. Diabetes 2001;50:921-7. (Pubitemid 32374520)
114. Shield JP, Gardner RJ, Wadsworth EJ, Whiteford ML, James RS, Robinson DO, et al. Aetiopathology and genetic basis of neonatal diabetes. Arch Dis Child Fetal Neonatal Ed 1997;76: F39-42. (Pubitemid 28234550)
115. Sperling MA. ATP-sensitive potassium channels-neonatal diabetes mellitus and beyond. N Engl J Med 2006;355:507-10. (Pubitemid 44162279)
116. Stanik J, Gasperikova D, Paskova M, Barak L, Javorkova J, Jancova E, et al. Prevalence of permanent neonatal diabetes in Slovakia and successful replacement of insulin with sulfonylurea therapy in KCNJ11 and ABCC8 mutation carriers. J Clin Endocrinol Metab 2007;92:1276-82. (Pubitemid 46556398)
117. Wiedemann B, Schober E, Waldhoer T, Koehle J, Flanagan SE, Mackay DJ, et al. Incidence of neonatal diabetes in Austria-calculation based on the Austrian Diabetes Register. Pediatr Diabetes 2010;11:18-23.
118. Slingerland AS, Shields BM, Flanagan SE, Bruining GJ, Noordam K, Gach A, et al. Referral rates for diagnostic testing support an incidence of permanent neonatal diabetes in three European countries of at least 1 in 260 000 live births. Diabetologia 2009;52:1683-5.
119. Temple IK, James RS, Crolla JA, Sitch FL, Jacobs PA, Howell WM, et al. An imprinted gene (s) for diabetes? Nat Genet 1995;9:110-2.
120. Mackay DJ, Coupe AM, Shield JP, Storr JN, Temple IK, Robinson DO. Relaxation of imprinted expression of ZAC and HYMAI in a patient with transient neonatal diabetes mellitus. Hum Genet 2002;110:139-44. (Pubitemid 36067430)
121. Garin I, Edghill EL, Akerman I, Rubio-Cabezas O, Rica I, Locke JM, et al. Recessive mutations in the INS gene result in neonatal diabetes through reduced insulin biosynthesis. Proc Natl Acad Sci U S A 2010;107:3105-10.
122. Ahamed A, Unnikrishnan AG, Pendsey SS, Nampoothiri S, Bhavani N, Praveen VP, et al. Permanent neonatal diabetes mellitus due to a C96Y heterozygous mutation in the insulin gene. A case report. JOP 2008;9:715-8.
123. Molven A, Ringdal M, Nordbo AM, Raeder H, Stoy J, Lipkind GM, et al. Mutations in the insulin gene can cause MODY and autoantibody-negative type 1 diabetes. Diabetes 2008;57:1131-5.
124. Polak M, Dechaume A, Cave H, Nimri R, Crosnier H, Sulmont V, et al. Heterozygous missense mutations in the insulin gene are linked to permanent diabetes appearing in the neonatal period or in early infancy: a report from the French ND (Neonatal Diabetes) Study Group. Diabetes 2008;57:1115-9.
125. Edghill EL, Flanagan SE, Patch AM, Boustred C, Parrish A, Shields B, et al. Insulin mutation screening in 1044 patients with diabetes: mutations in the INS gene are a common cause of neonatal diabetes but a rare cause of diabetes diagnosed in childhood or adulthood. Diabetes 2008;57:1034-42.
126. Stoy J, Edghill EL, Flanagan SE, Ye H, Paz VP, Pluzhnikov A, et al. Insulin gene mutations as a cause of permanent neonatal diabetes. Proc Natl Acad Sci U S A 2007;104:15040-4. (Pubitemid 47619589)
127. Stoffers DA, Zinkin NT, Stanojevic V, Clarke WL, Habener JF. Pancreatic agenesis attributable to a single nucleotide deletion in the human IPF1 gene coding sequence. Nat Genet 1997;15:106-10. (Pubitemid 27014961)
128. Schwitzgebel VM, Mamin A, Brun T, Ritz-Laser B, Zaiko M, Maret A, et al. Agenesis of human pancreas due to decreased half-life of insulin promoter factor 1. J Clin Endocrinol Metab 2003;88:4398-406. (Pubitemid 37153749)
129. Thomas IH, Saini NK, Adhikari A, Lee JM, Kasa-Vubu JZ, Vazquez DM, et al. Neonatal diabetes mellitus with pancreatic agenesis in an infant with homozygous IPF-1 Pro63fsX60 mutation. Pediatr Diabetes 2009;10:492-6.
130. Borowiec M, Antosik K, Fendler W, Deja G, Jarosz-Chobot P, Mysliwiec M, et al. Novel glucokinase mutations in patients with monogenic diabetes - clinical outline of gck-md and potential for founder effect in slavic population. Clin Genet 2011 Feb. 23. doi: 10.1111/j.1399-0004.2011.01656.x
131. Hussain K. Mutations in pancreatic ss-cell Glucokinase as a cause of hyperinsulinaemic hypoglycaemia and neonatal diabetes mellitus. Rev Endocr Metab Disord 2010;11:179-83.
132. Barbetti F, Cobo-Vuilleumier N, Dionisi-Vici C, Toni S, Ciampalini P, Massa O, et al. Opposite clinical phenotypes of glucokinase disease: Description of a novel activating mutation and contiguous inactivating mutations in human glucokinase (GCK) gene. Mol Endocrinol 2009;23:1983-9.
133. Osbak KK, Colclough K, Saint-Martin C, Beer NL, Bellanne-Chantelot C, Ellard S, et al. Update on mutations in glucokinase (GCK), which cause maturity-onset diabetes of the young, permanent neonatal diabetes, and hyperinsulinemic hypoglycemia. Hum Mutat 2009;30:1512-26.
134. Turkkahraman D, Bircan I, Tribble ND, Akcurin S, Ellard S, Gloyn AL. Permanent neonatal diabetes mellitus caused by a novel homo-zygous (T168A) glucokinase (GCK) mutation: initial response to oral sulphonylurea therapy. J Pediatr 2008;153:122-6.
135. Njolstad PR, Sagen JV, Bjorkhaug L, Odili S, Shehadeh N, Bakry D, et al. Permanent neonatal diabetes caused by glucokinase deficiency: inborn error of the glucose-insulin signaling pathway. Diabetes 2003;52:2854-60. (Pubitemid 37339717)
136. Gloyn AL. Glucokinase (GCK) mutations in hyper-and hypoglycemia: maturity-onset diabetes of the young, permanent neonatal diabetes, and hyperinsulinemia of infancy. Hum Mutat 2003;22:353-62. (Pubitemid 37346107)
137. Vaxillaire M, Samson C, Cave H, Metz C, Froguel P, Polak M. Glucokinase gene mutations are not a common cause of permanent neonatal diabetes in France. Diabetologia 2002;45:454-5. (Pubitemid 34267372)
138. Njolstad PR, Sovik O, Cuesta-Munoz A, Bjorkhaug L, Massa O, Barbetti F, et al. Neonatal diabetes mellitus due to complete gluco-kinase deficiency. N Engl J Med 2001;344:1588-92. (Pubitemid 32479911)
139. Rubio-Cabezas O, Diaz GF, Aragones A, Argente J, Campos-Barros A. Permanent neonatal diabetes caused by a homozygous nonsense mutation in the glucokinase gene. Pediatr Diabetes 2008;9:245-9. (Pubitemid 351785012)
140. Rubio-Cabezas O, Minton JA, Caswell R, Shield JP, Deiss D, Sumnik Z, et al. Clinical heterogeneity in patients with FOXP3 mutations presenting with permanent neonatal diabetes. Diabetes Care 2009;32:111-6.
141. Wildin RS, Ramsdell F, Peake J, Faravelli F, Casanova JL, Buist N, et al. X-linked neonatal diabetes mellitus, enteropathy and endo crino-pathy syndrome is the human equivalent of mouse scurfy. Nat Genet 2001;27:18-20. (Pubitemid 32044512)
142. ATP channel subunit Kir6.2 in mouse pancreatic beta cells recapitulates neonatal diabetes. J Clin Invest 2009;119:80-90.
143. + channel. Diabetes 2009;58:2419-24.
144. Villareal DT, Koster JC, Robertson H, Akrouh A, Miyake K, Bell GI, et al.
145. Chistiakov DA, Potapov VA, Khodirev DC, Shamkhalova MS, Shestakova MV, Nosikov VV. Genetic variations in the pancreatic ATP-sensi tive potassium channel, beta-cell dysfunction, and susceptibility to type 2 diabetes. Acta Diabetol 2009;46:43-9.
146. Riedel MJ, Steckley DC, Light PE. Current status of the E23K Kir6.2 polymorphism: implications for type-2 diabetes. Hum Genet 2005;116:133-45. (Pubitemid 40173997)
147. Riedel MJ, Boora P, Steckley D, de Vries G, Light PE. Kir6.2 polymorphisms sensitize beta-cell ATP-sensitive potassium channels to activation by acyl CoAs: a possible cellular mechanism for increased susceptibility to type 2 diabetes? Diabetes 2003;52:2630-5. (Pubitemid 37210554)
148. ATP channel subunits Kir6.2 (KCNJ11) and SUR1 (ABCC8) confirm that the KCNJ11 E23K variant is associated with type 2 diabetes. Diabetes 2003;52:568-72. (Pubitemid 36173218)
149. ATP channel subunits Kir6.2 (KCNJ11) and sulfonylurea receptor 1 (ABCC8) in diabetes mellitus and hyperinsulinism. Hum Mutat 2009;30:170-80.
150. ATP channelopathies in the pancreas. Pflugers Arch 2010;460:307-20.
151. Gloyn AL, Cummings EA, Edghill EL, Harries LW, 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. (Pubitemid 39071495)
152. Ioannou YS, Ellard S, Hattersley A, Skordis N. KCNJ11 activating mutations cause both transient and permanent neonatal diabetes mellitus in Cypriot patients. Pediatr Diabetes 2011;12:133-7.
153. Kochar IP, Kulkarni KP. Transient neonatal diabetes due to Kcnj11 mutation. Indian Pediatr 2010;47:359-60.
154. Khadilkar VV, Khadilkar AV, Kapoor RR, Hussain K, Hattersley AT, Ellard S. KCNJ11 activating mutation in an Indian family with remitting and relapsing diabetes. Indian J Pediatr 2010;77:551-4.
155. Stanik J, Lethby M, Flanagan SE, Gasperikova D, Milosovicova B, Lever M, et al. Coincidence of a novel KCNJ11 missense variant R365H with a paternally inherited 6q24 duplication in a patient with transient neonatal diabetes. Diabetes Care 2008;31:1736-7.
156. D'Amato E, Tammaro P, Craig TJ, Tosi A, Giorgetti R, Lorini R, et al. Variable phenotypic spectrum of diabetes mellitus in a family carrying a novel KCNJ11 gene mutation. Diabet Med 2008;25:651-6. (Pubitemid 351782829)
157. + channel genes cause transient neonatal diabetes and permanent diabetes in childhood or adulthood. Diabetes 2007;56:1930-7. (Pubitemid 47025462)
158. Yorifuji T, Nagashima K, Kurokawa K, Kawai M, Oishi M, Akazawa Y, et al. The C42R mutation in the Kir6.2 (KCNJ11) gene as a cause of transient neonatal diabetes, childhood diabetes, or later-onset, apparently type 2 diabetes mellitus. J Clin Endocrinol Metab 2005;90:3174-8. (Pubitemid 41014271)
159. Gloyn AL, Reimann F, Girard C, Edghill EL, Proks P, Pearson ER, et al. Relapsing diabetes can result from moderately activating mutations in KCNJ11. Hum Mol Genet 2005;14:925-34. (Pubitemid 40533103)
160. Zhou Q, Garin I, Castano L, Argente J, Munoz-Calvo MT, Perez de NG, et al. Neonatal diabetes caused by mutations in sulfonylurea receptor 1: interplay between expression and Mg-nucleotide gating defects of ATP-sensitive potassium channels. J Clin Endocrinol Metab 2010;95: E473-8.
161. de Wet H, Proks P, Lafond M, Aittoniemi J, Sansom MS, Flanagan SE, et al. A mutation (R826W) in nucleotide-binding domain 1 of ABCC8 reduces ATPase activity and causes transient neonatal diabetes. EMBO Rep 2008;9:648-54. (Pubitemid 351927685)
162. Vaxillaire M, Dechaume A, Busiah K, Cave H, Pereira S, Scharfmann R, et al. New ABCC8 mutations in relapsing neonatal diabetes and clinical features. Diabetes 2007;56:1737-41. (Pubitemid 46842623)
163. Babenko AP, Polak M, Cave H, Busiah K, Czernichow P, Scharfmann R, et al. Activating mutations in the ABCC8 gene in neonatal diabetes mellitus. N Engl J Med 2006;355:456-66. (Pubitemid 44162273)
164. Batra CM, Gupta N, Atwal G, Gupta V. Transient neonatal diabetes due to activating mutation in the ABCC8 gene encoding SUR1. Indian J Pediatr 2009;76:1169-72.
165. + channel genes cause tran sient neonatal diabetes and permanent diabetes in childhood or adulthood. Diabetes 2007;56:1930-7. (Pubitemid 47025462)
166. ATP channel cause transient neonatal diabetes, permanent neonatal diabetes or permanent diabetes diagnosed outside the neonatal period. Diabetes Obes Metab 2007;9:28-39. (Pubitemid 47543124)
167. Craig TJ, Shimomura K, Holl RW, Flanagan SE, Ellard S, Ashcroft FM. An in-frame deletion in Kir6.2 (KCNJ11) causing neonatal diabetes reveals a site of interaction between Kir6.2 and SUR1. J Clin Endo-crinol Metab 2009;94:2551-7.
168. ATP channel in glucose homeostasis in health and disease: more than meets the islet. J Physiol 2010;588:3201-9.
169. Ashcroft FM. ATP-sensitive potassium channelopathies: focus on insulin secretion. J Clin Invest 2005;115:2047-58. (Pubitemid 41134141)
170. Proks P, Antcliff JF, Lippiat J, Gloyn AL, Hattersley AT, Ashcroft FM. 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. (Pubitemid 39657439)
171. De Leon DD, Stanley CA. Permanent neonatal diabetes mellitus. 1993. In: Pagon RA, Bird TD, Dolan CR, Stephens K, editors. Gene Reviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2008 Feb. 08 [updated 2008 Mar. 04].
172. ATP channel mutation in neonatal diabetes is neuronal in origin. Science 2010;329:458-61.
173. Ashcroft FM. New uses for old drugs: neonatal diabetes and sulphonyl ureas. Cell Metab 2010;11:179-81.
174. Koster JC, Cadario F, Peruzzi C, Colombo C, Nichols CG, Barbetti F. The G53D mutation in Kir6.2 (KCNJ11) is associated with neonatal diabetes and motor dysfunction in adulthood that is improved with sulfonylurea therapy. J Clin Endocrinol Metab 2008;93:1054-61. (Pubitemid 351398592)
175. Pearson ER, Flechtner I, Njolstad PR, Malecki MT, Flanagan SE, Larkin B, et al. Switching from insulin to oral sulfonylureas in patients with diabetes due to Kir6.2 mutations. N Engl J Med 2006;355:467-77. (Pubitemid 44162274)
176. Zung A, Glaser B, Nimri R, Zadik Z. Glibenclamide treatment in permanent neonatal diabetes mellitus due to an activating mutation in Kir6.2. J Clin Endocrinol Metab 2004;89:5504-7. (Pubitemid 39518433)
177. Wambach JA, Marshall BA, Koster JC, White NH, Nichols CG. Success ful sulfonylurea treatment of an insulin-naive neonate with diabetes mellitus due to a KCNJ11 mutation. Pediatr Diabetes 2010;11:286-8.
178. Begum-Hasan J, Polychronakos C, Brill H. Familial permanent neonatal diabetes with KCNJ11 mutation and the response to glyburide therapy-a three-year follow-up. J Pediatr Endocrinol Metab 2008;21:895-903.
179. Klupa T, Skupien J, Mirkiewicz-Sieradzka B, Gach A, Noczynska A, Szalecki M, et al. Diabetic retinopathy in permanent neonatal diabetes due to Kir6.2 gene mutations: the results of a minimum 2-year follow-up after the transfer from insulin to sulphonylurea. Diabet Med 2009;26:663-4.
180. Mohamadi A, Clark LM, Lipkin PH, Mahone EM, Wodka EL, Plotnick LP. Medical and developmental impact of transition from subcutaneous insulin to oral glyburide in a 15-yr-old boy with neonatal diabetes mellitus and intermediate DEND syndrome: extend ing the age of KCNJ11 mutation testing in neonatal DM. Pediatr Diabetes 2010;11:203-7.
181. Stoy J, Greeley SA, Paz VP, Ye H, Pastore AN, Skowron KB, et al. Diagnosis and treatment of neonatal diabetes: a United States experience. Pediatr Diabetes 2008;9:450-9.
182. Slingerland AS, Nuboer R, Hadders-Algra M, Hattersley AT, Bruining GJ. Improved motor development and good long-term glycaemic control with sulfonylurea treatment in a patient with the syndrome of intermediate developmental delay, early-onset generalised epilepsy and neonatal diabetes associated with the V59M mutation in the KCNJ11 gene. Diabetologia 2006;49:2559-63.
183. Slingerland AS, Hurkx W, Noordam K, Flanagan SE, Jukema JW, Meiners LC, et al. Sulphonylurea therapy improves cognition in a patient with the V59M KCNJ11 mutation. Diabet Med 2008;25:277-81. (Pubitemid 351322710)
184. Masia R, Koster JC, Tumini S, Chiarelli F, Colombo C, Nichols CG, et al. An ATP-binding mutation (G334D) in KCNJ11 is associated with a sulfonylurea-insensitive form of developmental delay, epilepsy, and neonatal diabetes. Diabetes 2007;56:328-36.
185. Sumnik Z, Kolouskova S, Wales JK, Komarek V, Cinek O. Sulphonyl-urea treatment does not improve psychomotor development in children with KCNJ11 mutations causing permanent neonatal diabetes mellitus accompanied by developmental delay and epilepsy (DEND syndrome). Diabet Med 2007;24:1176-8. (Pubitemid 47437882)
186. Della MT, Battistim C, Radonsky V, Savoldelli RD, Damiani D, Kok F, et al. Glibenclamide unresponsiveness in a Brazilian child with permanent neonatal diabetes mellitus and DEND syndrome due to a C166Y mutation in KCNJ11 (Kir6.2) gene. Arq Bras Endocrinol Metabol 2008;52:1350-5.
187. Kumaraguru J, Flanagan SE, Greeley SA, Nuboer R, Stoy J, Philipson LH, et al. Tooth discoloration in patients with neonatal diabetes after transfer onto glibenclamide: a previously unreported side effect. Diabetes Care 2009;32:1428-30.
188. Codner E, Flanagan SE, Ugarte F, Garcia H, Vidal T, Ellard S, et al. Sulfonylurea treatment in young children with neonatal diabetes: dealing with hyperglycemia, hypoglycemia, and sick days. Diabetes Care 2007;30: e28-9. (Pubitemid 46684676)
189. Clark R, Proks P. ATP-sensitive potassium channels in health and disease. Adv Exp Med Biol 2010;654:165-92.
190. ATP-induced neonatal diabetes mellitus. Cell Metab 2009;9:140-51.
191. Hussain K. Congenital hyperinsulinism. Semin Fetal Neonatal Med 2005;10:369-76. (Pubitemid 40917324)
192. ATP channels. Pediatr Diabetes 2005;6:103-13. (Pubitemid 40916645)
193. Glaser B, Thornton P, Otonkoski T, Junien C. Genetics of neonatal hyper insulinism. Arch Dis Child Fetal Neonatal Ed 2000;82: F79-86.
194. Sempoux C, Guiot Y, Dubois D, Nollevaux MC, Saudubray JM, Nihoul-Fekete C, et al. Pancreatic beta-cell proliferation in persistent hyperin sulinemic hypoglycemia of infancy: an immunohistochemical study of 18 cases. Mod Pathol 1998;11:444-9. (Pubitemid 28224446)
195. Sempoux C, Guiot Y, Rahier J. The focal form of persistent hyperin-sulinemic hypoglycemia of infancy. Diabetes 2001;50: S182-3. (Pubitemid 32148250)
196. Sempoux C, Guiot Y, Jaubert F, Rahier J. Focal and diffuse forms of congenital hyperinsulinism: the keys for differential diagnosis. Endocr Pathol 2004;15:241-6. (Pubitemid 40169417)
197. Glaser B, Landau H, Permutt MA. Neonatal hyperinsulinism. Trends Endocrinol Metab 1999;10:55-61. (Pubitemid 29287823)
198. Fournet JC, Mayaud C, de Lonlay P, Verkarre V, Rahier J, Brunelle F, et al. Loss of imprinted genes and paternal SUR1 mutations lead to focal form of congenital hyperinsulinism. Horm Res 2000;53:2-6. (Pubitemid 30481313)
199. Fournet JC, Mayaud C, de Lonlay P, Gross-Morand MS, Verkarre V, Castanet M, et al. Unbalanced expression of 11p15 imprinted genes in focal forms of congenital hyperinsulinism: association with a reduction to homozygosity of a mutation in ABCC8 or KCNJ11. Am J Pathol 2001;158:2177-84. (Pubitemid 32545203)
200. Fournet JC, Junien C. Genetics of congenital hyperinsulinism. Endocr Pathol 2004;15:233-40.
201. de Lonlay P, Fournet JC, Touati G, Groos MS, Martin D, Sevin C, et al. Heterogeneity of persistent hyperinsulinaemic hypoglycaemia. A series of 175 cases. Eur J Pediatr 2002;161:37-48. (Pubitemid 34031274)
202. de Lonlay P, Fournet JC, Rahier J, Gross-Morand MS, Poggi-Travert F, Foussier V, et al. Somatic deletion of the imprinted 11p15 region in sporadic persistent hyperinsulinemic hypoglycemia of infancy is specific of focal adenomatous hyperplasia and endorses partial pancreatectomy. J Clin Invest 1997;100:802-7. (Pubitemid 27371697)
203. Glaser B, Ryan F, Donath M, Landau H, Stanley CA, Baker L, et al. Hyperinsulinism caused by paternal-specific inheritance of a recessive mutation in the sulfonylurea-receptor gene. Diabetes 1999;48:1652-7. (Pubitemid 29356876)
204. Verkarre V, Fournet JC, de Lonlay P, Gross-Morand MS, Devillers M, Rahier J, et al. Paternal mutation of the sulfonylurea receptor (SUR1) gene and maternal loss of 11p15 imprinted genes lead to persistent hyperinsulinism in focal adenomatous hyperplasia. J Clin Invest 1998;102:1286-91. (Pubitemid 28467556)
205. ATP channels and insulin secretion disorders. Am J Physiol Endocrinol Metab 2002;283: E207-16. (Pubitemid 34827434)
206. James C, Kapoor RR, Ismail D, Hussain K. The genetic basis of congenital hyperinsulinism. J Med Genet 2009;46:289-99.
207. Chan KW, Zhang H, Logothetis DE. N-terminal transmembrane domain of the SUR controls trafficking and gating of Kir6 channel subunits. EMBO J 2003;22:3833-43. (Pubitemid 36975711)
208. Montero LC, Pozo RJ, Munoz Calvo MT, Martos MG, Donoso MA, Rubio CO, et al. Hyperinsulinism-hyperammonemia syndrome due to a de novo mutation in exon 7 (G979A) of the glutamate dehydrogenase gene with excellent response to diazoxide. An Pediatr (Barc) 2004;61:433-7.
209. Yasuda K, Koda N, Kadowaki H, Ogawa Y, Kimura S, Kadowaki T, et al. A Japanese case of congenital hyperinsulinism with hyperammonemia due to a mutation in glutamate dehydrogenase (GLUD1) gene. Intern Med 2001;40:32-7. (Pubitemid 33613874)
210. Marquard J, Palladino AA, Stanley CA, Mayatepek E, Meissner T. Rare forms of congenital hyperinsulinism. Semin Pediatr Surg 2011;20:38-44.
211. Palladino AA, Stanley CA. The hyperinsulinism/hyperammonemia syndrome. Rev Endocr Metab Disord 2010;11:171-8.
212. Chik KK, Chan CW, Lam CW, Ng KL. Hyperinsulinism and hyper-ammonaemia syndrome due to a novel missense mutation in the allosteric domain of the glutamate dehydrogenase 1 gene. J Paediatr Child Health 2008;44:517-9.
213. de Lonlay P, Giurgea I, Sempoux C, Touati G, Jaubert F, Rahier J, et al. Dominantly inherited hyperinsulinaemic hypoglycaemia. J Inherit Metab Dis 2005;28:267-76. (Pubitemid 40723863)
214. Stanley CA. Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism. Mol Genet Metab 2004;81: S45-51. (Pubitemid 38402234)
215. Fujioka H, Okano Y, Inada H, Asada M, Kawamura T, Hase Y, et al. Molecular characterisation of glutamate dehydrogenase gene 780 defects in Japanese patients with congenital hyperinsulinism/hyper-ammonaemia. Eur J Hum Genet 2001;9:931-7. (Pubitemid 34145112)
216. Kelly A, Stanley CA. Disorders of glutamate metabolism. Ment Retard Dev Disabil Res Rev 2001;7:287-95. (Pubitemid 33108268)
217. Meissner T, Marquard J, Cobo-Vuilleumier N, Maringa M, Rodriguez-Bada P, Garcia-Gimeno MA, et al. Diagnostic difficulties in gluco-kinase hyperinsulinism. Horm Metab Res 2009;41:320-6.
218. Christesen HB, Tribble ND, Molven A, Siddiqui J, Sandal T, Brusgaard K, et al. Activating glucokinase (GCK) mutations as a cause of medically respon sive congenital hyperinsulinism: prevalence in children and characterisa tion of a novel GCK mutation. Eur J Endocrinol 2008;159:27-34. (Pubitemid 351969127)
219. Molven A, Matre GE, Duran M, Wanders RJ, Rishaug U, Njolstad PR, et al. Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation. Diabetes 2004;53:221-7. (Pubitemid 38044719)
220. Bas F, Darendeliler F, Demirkol D, Bundak R, Saka N, Gunoz H. Successful therapy with calcium channel blocker (nifedipine) in persistent neonatal hyperinsulinemic hypoglycemia of infancy. J Pediatr Endocrinol Metab 1999;12:873-8. (Pubitemid 30002276)
221. Eichmann D, Hufnagel M, Quick P, Santer R. Treatment of hyper-insulinaemic hypoglycaemia with nifedipine. Eur J Pediatr 1999;158:204-6.
222. Lindley KJ, Dunne MJ, Kane C, Shepherd RM, Squires PE, James RF, et al. Ionic control of beta cell function in nesidioblastosis. A possible therapeutic role for calcium channel blockade. Arch Dis Child 1996;74:373-8. (Pubitemid 26170040)
223. Shanbag P, Pathak A, Vaidya M, Shahid SK. Persistent hyper-insulinemic hypoglycemia of infancy-successful therapy with nifedi-pine. Indian J Pediatr 2002;69:271-2. (Pubitemid 34289036)
224. ATP channel-independent pathways. Diabetes 2001;50:329-39. (Pubitemid 32127290)