Unregulated insulin secretion by pancreatic beta cells in hyperinsulinism/hyperammonemia syndrome: Role of glutamate dehydrogenase, ATP-sensitive potassium channel, and nonselective cation channel

Pediatric Research

Volumn 59, Issue 3, 2006, Pages 359-364

  Kawajiri, Mie ^a   Okano, Yoshiyuki ^a   Kuno, Miyuki ^a   Tokuhara, Daisuke ^a   Hase, Yutaka ^b   Inada, Hiroshi ^a   Tashiro, Fumi ^c   Miyazaki, Jun Ichi ^c   Yamano, Tsunekazu ^a  

^a OSAKA CITY UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^b Department of Infectious Disease Control   (Japan)

^c OSAKA UNIVERSITY   (Japan)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE DIPHOSPHATE; ADENOSINE TRIPHOSPHATE; ADENOSINE TRIPHOSPHATE SENSITIVE POTASSIUM CHANNEL; CATION CHANNEL; GLUTAMATE DEHYDROGENASE; GUANOSINE TRIPHOSPHATE; INSULIN; ION CHANNEL; NONSELECTIVE CATION CHANNEL PROTEIN, RAT; POTASSIUM CHANNEL;

ALLOSTERISM; ANIMAL CELL; ARTICLE; CELL FUNCTION; CONTROLLED STUDY; ENZYME ACTIVITY; HYPERAMMONEMIA; HYPERINSULINISM; INSULIN RELEASE; MEMBRANE POTENTIAL; MISSENSE MUTATION; MOUSE; NONHUMAN; PANCREAS ISLET BETA CELL; PRIORITY JOURNAL; ANIMAL; CELL LINE; GENETICS; HUMAN; METABOLISM; PATCH CLAMP; SECRETION; SYNDROME;

ADENOSINE TRIPHOSPHATE; ANIMALS; CELL LINE; GLUTAMATE DEHYDROGENASE; HUMANS; HYPERAMMONEMIA; HYPERINSULINISM; INSULIN; INSULIN-SECRETING CELLS; ION CHANNELS; MICE; PATCH-CLAMP TECHNIQUES; POTASSIUM CHANNELS; SYNDROME;

EID: 33646097936     PISSN: 00313998     EISSN: None     Source Type: Journal    
DOI: 10.1203/01.pdr.0000198775.22719.46     Document Type: Article

References (30)

1. Zammarchi E, Filippi L, Novembre E, Donati MA 1996 Biochemical evaluation of a patient with a familial form of leucine-sensitive hypoglycemia and concomitant hyperammonemia. Metabolism 45:957-960
2. Weinzimer SA, Stanley CA, Berry GT, Yudkoff M, Tuchman M, Thomton PS 1997 A syndrome of congenital hyperinsulinism and hyperammonemia. J Pediatr 130:661-664
3. Stanley CA, Lieu YK, Hsu BY, Burlina AB, Greenberg CR, Hopwood NJ, Perlman K, Rich BH, Zammarchi E, Poncz M 1998 Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med 338:1352-1357
4. Stanley CA, Fang J, Kutyna K, Hsu BY, Ming JE, Glaser B, Poncz M 2000 Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. Diabetes 49:667-673
5. De Lonlay P, Benelli C, Fouque F, Ganguly A, Aral B, Dionisi-Vici C, Touati G, Heinrichs C, Rabier D, Kamoun P, Robert JJ, Stanley C, Saudubray JM 2001 Hyperinsulinism and hyperammonemia syndrome: report of twelve unrelated patients. Pediatr Res 50:353-357
6. Miki Y, Taki T, Ohura T, Kato H, Yanagisawa M, Hayashi Y 2000 Novel missense mutations in the glutamate dehydrogenase gene in the congenital hyperinsulinism hyperammonemia syndrome. J Pediatr 136:69-72
7. Fujioka H, Okano Y, Inada H, Asada M, Kawamura T, Hase Y, Yamano T 2001 Molecular characterisation of glutamate dehydrogenase gene defects in Japanese patients with congenital hyperinsulinism/hyperammonaemia. Eur J Hum Genet 9:931-937
8. Yorifuji T, Muroi J, Uematsu A, Hiramatsu H, Momoi T 1999 Hyperinsulinism-hyperammonemia syndrome caused by mutant glutamate dehydrogenase accompanied by novel enzyme kinetics. Hum Genet 104:476-479
9. Amuro N, Goto Y, Okazaki T 1990 Isolation and characterization of the two distinct genes for human glutamate dehydrogenase. Biochim Biophys Acta 1049:216-218
10. Tashiro F, Niwa H, Miyazaki J 1999 Constructing adenoviral vectors by using the circular form of the adenoviral genome cloned in a cosmid and the Cre-loxP recombination system. Hum Gene Ther 10:1845-1852
11. Miyazaki J, Araki K, Yamato E, Ikegami H, Asano T, Shibasaki Y, Oka Y, Yamamura K 1990 Establishment of a pancreatic beta cell line that retains glucose-inducible insulin secretion: special reference to expression of glucose transporter isoforms. Endocrinology 127:126-132
12. da Silva Xavier G, Varadi A, Ainscow EK, Rutter GA 2000 Regulation of gene expression by glucose in pancreatic beta-cells (MIN6) via insulin secretion and activation of phosphatidylinositol 3′-kinase. J Biol Chem 275:36269-36277
13. Tanizawa Y, Nakai K, Sasaki T, Anno T, Ohta Y, Inoue H, Matsuo K, Koga M, Furukawa S, Oka Y 2002 Unregulated elevation of glutamate dehydrogenase activity induces glutamine-stimulated insulin secretion: identification and characterization of a GLUD1 gene mutation and insulin secretion studies with MIN6 cells overexpressing the mutant glutamate dehydrogenase. Diabetes 51:712-717
14. + channels in MIN-6 cells. J Physiol 532:127-142
15. Qian F, Huang P, Ma L, Kuznetsov A, Tamarina N, Philipson LH 2002 TRP genes: candidates for nonselective cation channels and store-operated channels in insulin-secreting cells. Diabetes 51:S183-S189
16. 2+-sensitive nonselective cation channels in regulating the membrane potential of pancreatic beta-cells. Diabetes 47:1066-1073
17. Anno T, Uehara S, Katagiri H, Ohta Y, Ueda K, Mizuguchi H, Moriyama Y, Oka Y, Tanizawa Y 2004 Overexpression of constitutively activated glutamate dehydrogenase induces insulin secretion through enhanced glutamate oxidation. Am J Physiol Endocrinol Metab 286:E280-E285
18. Carobbio S, Ishihara H, Fernandez-Pascual S, Martin-Del-Rio R, Maeshler P 2004 Insulin secretion profiles are modified by overexpression of glutamate dehydrogenase in pancreatic islets. Diabetologia 47:266-276
19. Sakura H, Ammälä C, Smith PA, Gribble FM, Ashcroft FM 1995 Cloning and functional expression of the cDNA encoding a novel ATP-sensitive potassium channel subunit expressed in pancreatic beta-cells, brain, heart and skeletal muscle. FEBS Lett 377:338-344
20. Aguilar-Bryan L, Nichols CG, Wechsler SW, Clement JP 4th, Boyd AE 3rd, González G, Herrera-Sosa H, Nguy K, Bryan J, Nelson DA 1995 Cloning of the beta cell high-affinity sulfonylurea receptor: a regulator of insulin secretion. Science 268:423-426
21. Thomas P, Ye Y, Lightner E 1996 Mutation of the pancreatic islet inward rectifier Kir6.2 also leads to familial persistent hyperinsulinemic hypoglycemia of infancy. Hum Mol Genet 5:1809-1812
22. Gloyn AL, Pearson ER, Antcliff JF, Proks P, Bruining GJ, Slingerland AS, Howard N, Srinivasan S, Silva JM, Molnes J, Edghill EL, Frayling TM, Temple IK, Mackay D, Shield JP, Sumnik Z, van Rhijn A, Wales JK, Clark P, Gorman S, Aisenberg J, Ellard S, Njolstad PR, Ashcroft FM, Hattersley AT 2004 Activating mutations in the gene encoding the ATP-sensitive potassium-channel subunit Kir6.2 and permanent neonatal diabetes. N Engl J Med 350:1838-1849
23. ATP channel-deficient mice. Proc Natl Acad Sci U S A 95:10402-10406
24. Shiota C, Larsson O, Shelton KD, Shiota M, Efanov AM, Hoy M, Lindner J, Kooptiwut S, Juntti-Berggren L, Gromada J, Berggren PO, Magnuson MA 2002 Sulfonylurea receptor type 1 knock-out mice have intact feeding-stimulated insulin secretion despite marked impairment in their response to glucose. J Biol Chem 277:37176-37183
25. Sturgess NC, Hales CN, Ashford ML 1986 Inhibition of a calcium-activated, non-selective cation channel, in a rat insulinoma cell line, by adenine derivatives. FEBS Lett. 208:397-400
26. Suzuki M, Murata M, Ikeda M, Miyoshi T, Imai M 1998 Primary structure and functional expression of a novel non-selective cation channel. Biochem Biophys Res Commun 242:191-196
27. 2+. Cell Calcium 32:321-334
28. Leech CA, Habener JF 1997 Insulinotropic glucagon-like peptide-1-mediated activation of non-selective cation currents in insulinoma cells is mimicked by maitotoxin. J Biol Chem 272:17987-17993
29. 2+ stores in single rabbit portal vein myocytes. J Physiol 538:717-728
30. Reale V, Hales CN, Ashford ML 1994 Nucleotide Regulation of a calcium-activated cation channel in the rat insulinoma cell line, CRI-G1. J Membr Biol 141:101-112