Regulation of glutamate metabolism and insulin secretion by glutamate dehydrogenase in hypoglycemic children

American Journal of Clinical Nutrition

Volumn 90, Issue 3, 2009, Pages

  Stanley, Charles A ^a  

^a CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AMMONIA; GLUTAMATE DEHYDROGENASE; GLUTAMIC ACID; INWARDLY RECTIFYING POTASSIUM CHANNEL SUBUNIT KIR6.2; NEUROTRANSMITTER; VOLTAGE GATED CALCIUM CHANNEL; AMINO ACID; GUANOSINE TRIPHOSPHATE; INSULIN;

AMINO ACID METABOLISM; AMINO ACID SYNTHESIS; AMMONIA BLOOD LEVEL; AMMONIA FORMATION; BRAIN NERVE CELL; CONFERENCE PAPER; DIET RESTRICTION; GENE MUTATION; GENERALIZED EPILEPSY; GENETIC DISORDER; HUMAN; HYPERINSULINISM HYPERAMMONEMIA SYNDROME; HYPOGLYCEMIA; INSULIN RELEASE; LIVER CELL; METABOLIC DISORDER; MUTANT; NONHUMAN; PANCREAS ISLET BETA CELL; PROTEIN DEGRADATION; PROTEIN DIET; PROTEIN INTAKE; PROTEIN SYNTHESIS REGULATION; ANIMAL; BRAIN; ENZYMOLOGY; FEMALE; GENETICS; HYPERAMMONEMIA; MALE; METABOLISM; MOUSE; MUTATION; PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY; REVIEW; SECRETION;

MUS;

AMINO ACIDS; ANIMALS; BRAIN; DIETARY PROTEINS; FEMALE; GLUTAMATE DEHYDROGENASE; GLUTAMIC ACID; GUANOSINE TRIPHOSPHATE; HUMANS; HYPERAMMONEMIA; INSULIN; MALE; MICE; MUTATION; PERSISTENT HYPERINSULINEMIA HYPOGLYCEMIA OF INFANCY;

EID: 70349567012     PISSN: 00029165     EISSN: None     Source Type: Journal    
DOI: 10.3945/ajcn.2009.27462AA     Document Type: Conference Paper

References (45)

1. Fang J, Hsu BY, MacMullen CM, Poncz M, Smith TJ, Stanley CA. Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations. Biochem J 2002;363:81-87 (Pubitemid 34280615)
2. MacMullen C, Fang J, Hsu BYL, et al. Hyperinsulinism/hyperammonemia syndrome in children with regulatory mutations in the inhibitory GTP binding domain of glutamate dehydrogenase. J Clin Endocrinol Metab 2001;86:1782-1787 (Pubitemid 32374938)
3. Stanley CA, Lieu YK, Hsu BY, et al. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med 1998;338:1352-1357
4. Smith TJ, Peterson PE, Schmidt T, Fang J, Stanley CA. Structures of bovine glutamate dehydrogenase complexes elucidate the mechanism of purine regulation. J Mol Biol 2001;307:707-720 (Pubitemid 33027686)
5. Allen A, Kwagh J, Fang J, Stanley CA, Smith TJ. Evolution of glutamate dehydrogenase regulation of insulin homeostasis is an example of molecular exaptation. Biochemistry 2004;43:14431-14443 (Pubitemid 39491978)
6. Smith TJ, Schmidt T, Fang J, Wu J, Siuzdak G, Stanley CA. The structure of apo human glutamate dehydrogenase details subunit communication and allostery. J Mol Biol 2002;318:765-777 (Pubitemid 34729367)
7. Haigis MC, Mostoslavsky R, Haigis KM, et al. SIRT4 inhibits glutamate dehydrogenase and opposes the effects of calorie restriction in pancreatic beta cells. Cell 2006;126:941-954 (Pubitemid 44321935)
8. Li C, Allen A, Kwagh J, et al. Green tea polyphenols modulate insulin secretion by inhibiting glutamate dehydrogenase. J Biol Chem 2006;281:10214-10221 (Pubitemid 43864558)
9. Kibbey RG, Pongratz RL, Romanelli AJ, Wollheim CB, Cline GW, Shulman GI. Mitochondrial GTP regulates glucose-stimulated insulin secretion. Cell Metab 2007;5:253-264 (Pubitemid 46477853)
10. Bahi-Buisson N, Roze E, Dionisi C, et al. Neurological aspects in hyperinsulinism hyperammonemia syndrome. Dev Med Child Neurol 2008;50:945-949
11. Raizen DM, Brooks-Kayal A, Steinkrauss L, Tennekoon GI, Stanley CA, Kelly A. Central nervous system hyperexcitability associated with glutamate dehydrogenase gain of function mutations. J Pediatr 2005;146:388-394 (Pubitemid 40341751)
12. Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA. Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome. J Pediatr 2001;138:383-389 (Pubitemid 32207142)
13. Kelly A, Ng D, Ferry RJ Jr, et al. Acute insulin responses to leucine in children with the hyperinsulinism/hyperammonemia syndrome. J Clin Endocrinol Metab 2001;86:3724-3728 (Pubitemid 32755967)
14. Li C, Matter A, Kelly A, et al. Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice. J Biol Chem 2006;281:15064-15072 (Pubitemid 43855095)
15. Fourtner SH, Stanley CA, Kelly A. Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations. J Pediatr 2006;149:47-52. (Pubitemid 44062532)
16. Li C, Buettger C, Kwagh J, et al. A signaling role of glutamine in insulin secretion. J Biol Chem 2004;279:13393-13401 (Pubitemid 38468862)
17. Fernstrom JD. Introduction to the symposium. Am J Clin Nutr 2009;90(suppl):705S-6S.
18. Krebs JR. The gourmet ape: evolution and human food preferences. Am J Clin Nutr 2009;90(suppl):707S-11S.
19. Curtis RI. Umami and the foods of classical antiquity. Am J Clin Nutr 2009;90(suppl):712S-8S.
20. Kurihara K. Glutamate: from discovery as a food flavor to role as a basic taste (umami). Am J Clin Nutr 2009;90(suppl):719S-22S.
21. Beauchamp GK. Sensory and receptor responses to umami: an overview of pioneering work. Am J Clin Nutr 2009;90(suppl):723S-7S.
22. Sano C. History of glutamate production. Am J Clin Nutr 2009;90(suppl):728S-32S.
23. Li X. T1R receptors mediate mammalian sweet and umami taste. Am J Clin Nutr 2009;90(suppl):733S-7S.
24. Chaudhari N, Pereira E, Roper SD. Taste receptors for umami: the case for multiple receptors. Am J Clin Nutr 2009;90(suppl):738S-42S.
25. San Gabriel A, Maekawa T, Uneyama H, Torii K. Metabotropic glutamate receptor type 1 in taste tissue. Am J Clin Nutr 2009;90(suppl):743S-6S.
26. Yasumatsu K, Horio N, Murata Y, et al. Multiple receptors underlie glutamate taste responses in mice. Am J Clin Nutr 2009;90(suppl):747S-52S.
27. Kinnamon SC. Umami taste transduction mechanisms. Am J Clin Nutr 2009;90(suppl):753S-5S.
28. Bachmanov AA, Inoue M, Ji H, Murata Y, Tordoff MG, Beauchamp GK. Glutamate taste and appetite in laboratory mice: physiologic and genetic analyses. Am J Clin Nutr 2009;90(suppl):756S-63S.
29. Shigemura N, Shirosaki S, Ohkuri T, et al. Variation in umami perception and in candidate genes for the umami receptor in mice and humans. Am J Clin Nutr 2009;90(suppl):764S-9S.
30. Chen Q-Y, Alarcon S, Tharp A, et al. Perceptual variation in umami taste and polymorphisms in TAS1R taste receptor genes. Am J Clin Nutr 2009;90(suppl):770S-9S.
31. Mennella JA, Forestell CA, Morgan LK, Beauchamp GK. Early milk feeding influences taste acceptance and liking during infancy. Am J Clin Nutr 2009;90(suppl):780S-8S.
32. Raliou M, Wiencis A, Pillias A-M, et al. Nonsynonymous single nucleotide polymorphisms in human tas1r1, tas1r3, and mGluR1 and individual taste sensitivity to glutamate. Am J Clin Nutr 2009;90(suppl):789S-99S.
33. Donaldson LF, Bennett L, Baic S, Melichar JK. Taste and weight: is there a link? Am J Clin Nutr 2009;90(suppl):800S-3S.
34. Rolls ET. Functional neuroimaging of umami taste: what makes umami pleasant? Am J Clin Nutr 2009;90(suppl):804S-13S.
35. Blachier F, Boutry C, Bos C, Tomé D. Metabolism and functions of L-glutamate in the epithelial cells of the small and large intestines. Am J Clin Nutr 2009;90(suppl):814S-21S.
36. Kokrashvili Z, Mosinger B, Margolskee RF. Taste signaling elements expressed in gut enteroendocrine cells regulate nutrient-responsive secretion of gut hormones. Am J Clin Nutr 2009;90(suppl):822S-5S.
37. Akiba Y, Kaunitz JD. Luminal chemosensing and upper gastrointestinal mucosal defenses. Am J Clin Nutr 2009;90(suppl):826S-31S.
38. Kondoh T, Mallick HN, Torii K. Activation of the gut-brain axis by dietary glutamate and physiologic significance in energy homeostasis. Am J Clin Nutr 2009;90(suppl):832S-7S.
39. Tomé D, Schwarz J, Fromentin G, Darcel N. Protein, amino acids, vagus nerve signaling, and the brain. Am J Clin Nutr 2009;90(suppl):838S-43S.
40. Yamamoto S, Tomoe M, Toyama K, Kawai M, Uneyama H. Can dietary supplementation of monosodium glutamate improve the health of the elderly? Am J Clin Nutr 2009;90(suppl):844S-9S.
41. Burrin DG, Stoll B. Metabolic fate and function of dietary glutamate in the gut. Am J Clin Nutr 2009;90(suppl):850S-6S.
42. Brosnan ME, Brosnan JT. Hepatic glutamate metabolism: a tale of 2 hepatocytes. Am J Clin Nutr 2009;90(suppl):857S-61S.
43. Hawkins RA. The blood-brain barrier and glutamate. Am J Clin Nutr 2009;90(suppl):867S-74S.
44. Magistretti PJ. Role of glutamate in neuron-glia metabolic coupling. Am J Clin Nutr 2009;90(suppl):875S-80S.
45. Fernstrom JD. Symposium summary. Am J Clin Nutr 2009;90(suppl):881S-5S.