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

Stanley CA.

Am J Clin Nutr. 2009 Sep;90(3):862S-866S. Epub 2009 Jul 22. Review.PMID: 19625687 [PubMed - indexed for MEDLINE]Related articles

Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism.

Laje P, Halaby L, Adzick NS, Stanley CA.

Pediatr Diabetes. 2009 Jun 25. [Epub ahead of print]PMID: 19558634 [PubMed - as supplied by publisher]Related articles

[Hyperinsulinism in infancy and childhood: when an insulin level is not always enough]

Palladino AA, Bennett MJ, Stanley CA.

Ann Biol Clin (Paris). 2009 May-Jun;67(3):245-54. French. PMID: 19411227 [PubMed]Related articles

Extremes of clinical and enzymatic phenotypes in children with hyperinsulinism caused by glucokinase activating mutations.

Sayed S, Langdon DR, Odili S, Chen P, Buettger C, Schiffman AB, Suchi M, Taub R, Grimsby J, Matschinsky FM, Stanley CA.

Diabetes. 2009 Jun;58(6):1419-27. Epub 2009 Mar 31.PMID: 19336674 [PubMed - indexed for MEDLINE]Related articlesFree article

Sulfonylurea receptor 1 mutations that cause opposite insulin secretion defects with chemical chaperone exposure.

Pratt EB, Yan FF, Gay JW, Stanley CA, Shyng SL.

J Biol Chem. 2009 Mar 20;284(12):7951-9. Epub 2009 Jan 16.PMID: 19151370 [PubMed - indexed for MEDLINE]Related articles

Neurological aspects in hyperinsulinism-hyperammonaemia syndrome.

Kelly A, Stanley CA.

Dev Med Child Neurol. 2008 Dec;50(12):888. No abstract available. PMID: 19046183 [PubMed - indexed for MEDLINE]Related articles

Follow-up analysis of genome-wide association data identifies novel loci for type 1 diabetes.

Grant SF, Qu HQ, Bradfield JP, Marchand L, Kim CE, Glessner JT, Grabs R, Taback SP, Frackelton EC, Eckert AW, Annaiah K, Lawson ML, Otieno FG, Santa E, Shaner JL, Smith RM, Skraban R, Imielinski M, Chiavacci RM, Grundmeier RW, Stanley CA, Kirsch SE, Waggott D, Paterson AD, Monos DS; DCCT/EDIC Research Group, Polychronakos C, Hakonarson H.

Diabetes. 2009 Jan;58(1):290-5. Epub 2008 Oct 7.PMID: 18840781 [PubMed - indexed for MEDLINE]Related articlesFree article

Untangling the glutamate dehydrogenase allosteric nightmare.

Smith TJ, Stanley CA.

Trends Biochem Sci. 2008 Nov;33(11):557-64. Epub 2008 Sep 24. Review.PMID: 18819805 [PubMed - indexed for MEDLINE]Related articles

Poor specificity of low growth hormone and cortisol levels during fasting hypoglycemia for the diagnoses of growth hormone deficiency and adrenal insufficiency.

Kelly A, Tang R, Becker S, Stanley CA.

Pediatrics. 2008 Sep;122(3):e522-8. Epub 2008 Aug 11.PMID: 18694902 [PubMed - indexed for MEDLINE]Related articlesFree article

Exendin-(9-39) corrects fasting hypoglycemia in SUR-1-/- mice by lowering cAMP in pancreatic beta-cells and inhibiting insulin secretion.

De León DD, Li C, Delson MI, Matschinsky FM, Stanley CA, Stoffers DA.

J Biol Chem. 2008 Sep 19;283(38):25786-93. Epub 2008 Jul 17.PMID: 18635551 [PubMed - indexed for MEDLINE]Related articlesFree article

Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations.

Pinney SE, MacMullen C, Becker S, Lin YW, Hanna C, Thornton P, Ganguly A, Shyng SL, Stanley CA.

J Clin Invest. 2008 Aug;118(8):2877-86.PMID: 18596924 [PubMed - indexed for MEDLINE]Related articlesFree article

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.

Li C, Nissim I, Chen P, Buettger C, Najafi H, Daikhin Y, Nissim I, Collins HW, Yudkoff M, Stanley CA, Matschinsky FM.

J Biol Chem. 2008 Jun 20;283(25):17238-49. Epub 2008 Apr 29.PMID: 18445600 [PubMed - indexed for MEDLINE]Related articlesFree article

Destabilization of ATP-sensitive potassium channel activity by novel KCNJ11 mutations identified in congenital hyperinsulinism.

Lin YW, Bushman JD, Yan FF, Haidar S, MacMullen C, Ganguly A, Stanley CA, Shyng SL.

J Biol Chem. 2008 Apr 4;283(14):9146-56. Epub 2008 Feb 4.PMID: 18250167 [PubMed - indexed for MEDLINE]Related articlesFree article

A novel susceptibility locus for type 1 diabetes on Chr12q13 identified by a genome-wide association study.

Hakonarson H, Qu HQ, Bradfield JP, Marchand L, Kim CE, Glessner JT, Grabs R, Casalunovo T, Taback SP, Frackelton EC, Eckert AW, Annaiah K, Lawson ML, Otieno FG, Santa E, Shaner JL, Smith RM, Onyiah CC, Skraban R, Chiavacci RM, Robinson LJ, Stanley CA, Kirsch SE, Devoto M, Monos DS, Grant SF, Polychronakos C.

Diabetes. 2008 Apr;57(4):1143-6. Epub 2008 Jan 15.PMID: 18198356 [PubMed - indexed for MEDLINE]Related articlesFree article

Hyperinsulinism in infancy and childhood: when an insulin level is not always enough.

Palladino AA, Bennett MJ, Stanley CA.

Clin Chem. 2008 Feb;54(2):256-63. Epub 2007 Dec 21. Review.PMID: 18156285 [PubMed - indexed for MEDLINE]Related articlesFree article

The impact of Juvenile Huntington's Disease on the family: the case of a rare childhood condition.

Brewer HM, Eatough V, Smith JA, Stanley CA, Glendinning NW, Quarrell OW.

J Health Psychol. 2008 Jan;13(1):5-16.PMID: 18086713 [PubMed - indexed for MEDLINE]Related articles

Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism.

Hardy OT, Hernandez-Pampaloni M, Saffer JR, Scheuermann JS, Ernst LM, Freifelder R, Zhuang H, MacMullen C, Becker S, Adzick NS, Divgi C, Alavi A, Stanley CA.

J Clin Endocrinol Metab. 2007 Dec;92(12):4706-11. Epub 2007 Sep 25.PMID: 17895314 [PubMed - indexed for MEDLINE]Related articlesFree article

A genome-wide association study identifies KIAA0350 as a type 1 diabetes gene.

Hakonarson H, Grant SF, Bradfield JP, Marchand L, Kim CE, Glessner JT, Grabs R, Casalunovo T, Taback SP, Frackelton EC, Lawson ML, Robinson LJ, Skraban R, Lu Y, Chiavacci RM, Stanley CA, Kirsch SE, Rappaport EF, Orange JS, Monos DS, Devoto M, Qu HQ, Polychronakos C.

Nature. 2007 Aug 2;448(7153):591-4. Epub 2007 Jul 15.PMID: 17632545 [PubMed - indexed for MEDLINE]Related articles

Congenital hyperinsulinism associated ABCC8 mutations that cause defective trafficking of ATP-sensitive K+ channels: identification and rescue.

Yan FF, Lin YW, MacMullen C, Ganguly A, Stanley CA, Shyng SL.

Diabetes. 2007 Sep;56(9):2339-48. Epub 2007 Jun 15.PMID: 17575084 [PubMed - indexed for MEDLINE]Related articlesFree article

A mutation in the TMD0-L0 region of sulfonylurea receptor-1 (L225P) causes permanent neonatal diabetes mellitus (PNDM).

Masia R, De Leon DD, MacMullen C, McKnight H, Stanley CA, Nichols CG.

Diabetes. 2007 May;56(5):1357-62. Epub 2007 Feb 22.PMID: 17317760 [PubMed - indexed for MEDLINE]Related articlesFree article

Caring for a child with Juvenile Huntington's Disease: helpful and unhelpful support.

Brewer HM, Smith JA, Eatough V, Stanley CA, Glendinning NW, Quarrell OW.

J Child Health Care. 2007 Mar;11(1):40-52.PMID: 17287223 [PubMed - indexed for MEDLINE]Related articles

Hypoglycemia in the neonate.

Stanley CA.

Pediatr Endocrinol Rev. 2006 Dec;4 Suppl 1:76-81. Review.PMID: 17261974 [PubMed - indexed for MEDLINE]Related articles

Diagnosis and localization of focal congenital hyperinsulinism by 18F-fluorodopa PET scan.

Hardy OT, Hernandez-Pampaloni M, Saffer JR, Suchi M, Ruchelli E, Zhuang H, Ganguly A, Freifelder R, Adzick NS, Alavi A, Stanley CA.

J Pediatr. 2007 Feb;150(2):140-5.PMID: 17236890 [PubMed - indexed for MEDLINE]Related articles

Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomography scan in an infant with congenital hyperinsulinism.

Peranteau WH, Bathaii SM, Pawel B, Hardy O, Alavi A, Stanley CA, Adzick NS.

J Pediatr Surg. 2007 Jan;42(1):188-92.PMID: 17208563 [PubMed - indexed for MEDLINE]Related articles

Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates.

De León DD, Stanley CA.

Nat Clin Pract Endocrinol Metab. 2007 Jan;3(1):57-68. Review.PMID: 17179930 [PubMed - indexed for MEDLINE]Related articles

Prenatal diagnosis and postnatal management of diffuse congenital hyperinsulinism: a case report.

Peranteau WH, Ganguly A, Steinmuller L, Thornton P, Johnson MP, Howell LJ, Stanley CA, Adzick NS.

Fetal Diagn Ther. 2006;21(6):515-8. Epub 2006 Sep 12.PMID: 16969006 [PubMed - indexed for MEDLINE]Related articles

Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations.

Fourtner SH, Stanley CA, Kelly A.

J Pediatr. 2006 Jul;149(1):47-52.PMID: 16860127 [PubMed - indexed for MEDLINE]Related articles

Parsing ketotic hypoglycaemia.

Stanley CA.

Arch Dis Child. 2006 Jun;91(6):460-1. No abstract available. PMID: 16714717 [PubMed - indexed for MEDLINE]Related articlesFree article

The personal experience of juvenile Huntington's disease: an interpretative phenomenological analysis of parents' accounts of the primary features of a rare genetic condition.

Smith JA, Brewer HM, Eatough V, Stanley CA, Glendinning NW, Quarrell OW.

Clin Genet. 2006 Jun;69(6):486-96.PMID: 16712700 [PubMed - indexed for MEDLINE]Related articles

The diagnosis of ectopic focal hyperinsulinism of infancy with [18F]-dopa positron emission tomography.

Hussain K, Seppänen M, Näntö-Salonen K, Adzick NS, Stanley CA, Thornton P, Minn H.

J Clin Endocrinol Metab. 2006 Aug;91(8):2839-42. Epub 2006 May 9.PMID: 16684819 [PubMed - indexed for MEDLINE]Related articlesFree article

Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice.

Li C, Matter A, Kelly A, Petty TJ, Najafi H, MacMullen C, Daikhin Y, Nissim I, Lazarow A, Kwagh J, Collins HW, Hsu BY, Nissim I, Yudkoff M, Matschinsky FM, Stanley CA.

J Biol Chem. 2006 Jun 2;281(22):15064-72. Epub 2006 Mar 30.PMID: 16574664 [PubMed - indexed for MEDLINE]Related articlesFree article

Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism.

Hoe FM, Thornton PS, Wanner LA, Steinkrauss L, Simmons RA, Stanley CA.

J Pediatr. 2006 Feb;148(2):207-12.PMID: 16492430 [PubMed - indexed for MEDLINE]Related articles

Green tea polyphenols modulate insulin secretion by inhibiting glutamate dehydrogenase.

Li C, Allen A, Kwagh J, Doliba NM, Qin W, Najafi H, Collins HW, Matschinsky FM, Stanley CA, Smith TJ.

J Biol Chem. 2006 Apr 14;281(15):10214-21. Epub 2006 Feb 13.PMID: 16476731 [PubMed - indexed for MEDLINE]Related articlesFree article

Medications used in the treatment of hypoglycemia due to congenital hyperinsulinism of infancy (HI).

Hussain K, Aynsley-Green A, Stanley CA.

Pediatr Endocrinol Rev. 2004 Nov;2 Suppl 1:163-7. Review.PMID: 16456495 [PubMed - indexed for MEDLINE]Related articles

Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism.

Suchi M, MacMullen CM, Thornton PS, Adzick NS, Ganguly A, Ruchelli ED, Stanley CA.

Mod Pathol. 2006 Jan;19(1):122-9.PMID: 16357843 [PubMed - indexed for MEDLINE]Related articlesFree article

A novel KCNJ11 mutation associated with congenital hyperinsulinism reduces the intrinsic open probability of beta-cell ATP-sensitive potassium channels.

Lin YW, MacMullen C, Ganguly A, Stanley CA, Shyng SL.

J Biol Chem. 2006 Feb 3;281(5):3006-12. Epub 2005 Dec 6.PMID: 16332676 [PubMed - indexed for MEDLINE]Related articlesFree article

Effects of hypoglycemia on developmental outcome in children with congenital hyperinsulinism.

Steinkrauss L, Lipman TH, Hendell CD, Gerdes M, Thornton PS, Stanley CA.

J Pediatr Nurs. 2005 Apr;20(2):109-18.PMID: 15815570 [PubMed - indexed for MEDLINE]Related articles

Central nervous system hyperexcitability associated with glutamate dehydrogenase gain of function mutations.

Raizen DM, Brooks-Kayal A, Steinkrauss L, Tennekoon GI, Stanley CA, Kelly A.

J Pediatr. 2005 Mar;146(3):388-94.PMID: 15756227 [PubMed - indexed for MEDLINE]Related articles

Insights into the structure and regulation of glucokinase from a novel mutation (V62M), which causes maturity-onset diabetes of the young.

Gloyn AL, Odili S, Zelent D, Buettger C, Castleden HA, Steele AM, Stride A, Shiota C, Magnuson MA, Lorini R, d'Annunzio G, Stanley CA, Kwagh J, van Schaftingen E, Veiga-da-Cunha M, Barbetti F, Dunten P, Han Y, Grimsby J, Taub R, Ellard S, Hattersley AT, Matschinsky FM.

J Biol Chem. 2005 Apr 8;280(14):14105-13. Epub 2005 Jan 25.PMID: 15677479 [PubMed - indexed for MEDLINE]Related articlesFree article

Carnitine deficiency disorders in children.

Stanley CA.

Ann N Y Acad Sci. 2004 Nov;1033:42-51. Review.PMID: 15591002 [PubMed - indexed for MEDLINE]Related articles

Genotype-phenotype correlations in children with congenital hyperinsulinism due to recessive mutations of the adenosine triphosphate-sensitive potassium channel genes.

Henwood MJ, Kelly A, Macmullen C, Bhatia P, Ganguly A, Thornton PS, Stanley CA.

J Clin Endocrinol Metab. 2005 Feb;90(2):789-94. Epub 2004 Nov 23.PMID: 15562009 [PubMed - indexed for MEDLINE]Related articlesFree article

Evolution of glutamate dehydrogenase regulation of insulin homeostasis is an example of molecular exaptation.

Allen A, Kwagh J, Fang J, Stanley CA, Smith TJ.

Biochemistry. 2004 Nov 16;43(45):14431-43.PMID: 15533048 [PubMed - indexed for MEDLINE]Related articles

Blood glucose control during selective arterial stimulation and venous sampling for localization of focal hyperinsulinism lesions in anesthetized children.

Cucchiaro G, Markowitz SD, Kaye R, Adzick NS, Litman RS, Stanley CA, Watcha MF.

Anesth Analg. 2004 Oct;99(4):1044-8, table of contents.PMID: 15385347 [PubMed - indexed for MEDLINE]Related articlesFree article

Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the extent of pancreatectomy.

Suchi M, Thornton PS, Adzick NS, MacMullen C, Ganguly A, Stanley CA, Ruchelli ED.

Am J Surg Pathol. 2004 Oct;28(10):1326-35.PMID: 15371948 [PubMed - indexed for MEDLINE]Related articles

Familial leucine-sensitive hypoglycemia of infancy due to a dominant mutation of the beta-cell sulfonylurea receptor.

Magge SN, Shyng SL, MacMullen C, Steinkrauss L, Ganguly A, Katz LE, Stanley CA.

J Clin Endocrinol Metab. 2004 Sep;89(9):4450-6.PMID: 15356046 [PubMed - indexed for MEDLINE]Related articlesFree article

Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism.

Stanley CA.

Mol Genet Metab. 2004 Apr;81 Suppl 1:S45-51. Review.PMID: 15050973 [PubMed - indexed for MEDLINE]Related articles

A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy.

Adzick NS, Thornton PS, Stanley CA, Kaye RD, Ruchelli E.

J Pediatr Surg. 2004 Mar;39(3):270-5.PMID: 15017536 [PubMed - indexed for MEDLINE]Related articles

A signaling role of glutamine in insulin secretion.

Li C, Buettger C, Kwagh J, Matter A, Daikhin Y, Nissim IB, Collins HW, Yudkoff M, Stanley CA, Matschinsky FM.

J Biol Chem. 2004 Apr 2;279(14):13393-401. Epub 2004 Jan 20.PMID: 14736887 [PubMed - indexed for MEDLINE]Related articlesFree article

Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation.

Stanley CA, Thornton PS, Ganguly A, MacMullen C, Underwood P, Bhatia P, Steinkrauss L, Wanner L, Kaye R, Ruchelli E, Suchi M, Adzick NS.

J Clin Endocrinol Metab. 2004 Jan;89(1):288-96. Erratum in: J Clin Endocrinol Metab. 2005 Jan;90(1):189. PMID: 14715863 [PubMed - indexed for MEDLINE]Related articlesFree article

Histopathology of congenital hyperinsulinism: retrospective study with genotype correlations.

Suchi M, MacMullen C, Thornton PS, Ganguly A, Stanley CA, Ruchelli ED.

Pediatr Dev Pathol. 2003 Jul-Aug;6(4):322-33.PMID: 14692646 [PubMed - indexed for MEDLINE]Related articles