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Results: 1 to 20 of 33

1.

On the reversibility of glutamate dehydrogenase and the source of hyperammonemia in the hyperinsulinism/hyperammonemia syndrome.

Treberg JR, Brosnan ME, Watford M, Brosnan JT.

Adv Enzyme Regul. 2010;50(1):34-43. doi: 10.1016/j.advenzreg.2009.10.029. Epub 2009 Nov 4. Review. No abstract available.

PMID:
19895831
[PubMed - indexed for MEDLINE]
2.

3-Hydroxyacyl-coenzyme A dehydrogenase deficiency and hyperinsulinemic hypoglycemia: characterization of a novel mutation and severe dietary protein sensitivity.

Kapoor RR, James C, Flanagan SE, Ellard S, Eaton S, Hussain K.

J Clin Endocrinol Metab. 2009 Jul;94(7):2221-5. doi: 10.1210/jc.2009-0423. Epub 2009 May 5.

PMID:
19417036
[PubMed - indexed for MEDLINE]
3.

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. doi: 10.1074/jbc.M804372200. Epub 2008 Jul 17.

PMID:
18635551
[PubMed - indexed for MEDLINE]
Free PMC Article
4.

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. doi: 10.1074/jbc.M709235200. Epub 2008 Apr 29.

PMID:
18445600
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency.

Filling C, Keller B, Hirschberg D, Marschall HU, Jörnvall H, Bennett MJ, Oppermann U.

Biochem Biophys Res Commun. 2008 Mar 28;368(1):6-11. Epub 2007 Nov 26.

PMID:
18036338
[PubMed - indexed for MEDLINE]
6.

Specificity in beta cell expression of L-3-hydroxyacyl-CoA dehydrogenase, short chain, and potential role in down-regulating insulin release.

Martens GA, Vervoort A, Van de Casteele M, Stangé G, Hellemans K, Van Thi HV, Schuit F, Pipeleers D.

J Biol Chem. 2007 Jul 20;282(29):21134-44. Epub 2007 May 9.

PMID:
17491019
[PubMed - indexed for MEDLINE]
Free Article
7.

Functional genomics of the beta-cell: short-chain 3-hydroxyacyl-coenzyme A dehydrogenase regulates insulin secretion independent of K+ currents.

Hardy OT, Hohmeier HE, Becker TC, Manduchi E, Doliba NM, Gupta RK, White P, Stoeckert CJ Jr, Matschinsky FM, Newgard CB, Kaestner KH.

Mol Endocrinol. 2007 Mar;21(3):765-73. Epub 2006 Dec 21.

PMID:
17185391
[PubMed - indexed for MEDLINE]
8.

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]
9.

SIRT4 inhibits glutamate dehydrogenase and opposes the effects of calorie restriction in pancreatic beta cells.

Haigis MC, Mostoslavsky R, Haigis KM, Fahie K, Christodoulou DC, Murphy AJ, Valenzuela DM, Yancopoulos GD, Karow M, Blander G, Wolberger C, Prolla TA, Weindruch R, Alt FW, Guarente L.

Cell. 2006 Sep 8;126(5):941-54.

PMID:
16959573
[PubMed - indexed for MEDLINE]
Free Article
10.

Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase.

Bennett MJ, Russell LK, Tokunaga C, Narayan SB, Tan L, Seegmiller A, Boriack RL, Strauss AW.

Mol Genet Metab. 2006 Sep-Oct;89(1-2):74-9. Epub 2006 May 24.

PMID:
16725361
[PubMed - indexed for MEDLINE]
11.

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]
Free Article
12.

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]
Free Article
13.

Hyperinsulinism of infancy associated with a novel splice site mutation in the SCHAD gene.

Hussain K, Clayton PT, Krywawych S, Chatziandreou I, Mills P, Ginbey DW, Geboers AJ, Berger R, van den Berg IE, Eaton S.

J Pediatr. 2005 May;146(5):706-8.

PMID:
15870679
[PubMed - indexed for MEDLINE]
14.

Mass spectrometry and the search for moonlighting proteins.

Jeffery CJ.

Mass Spectrom Rev. 2005 Nov-Dec;24(6):772-82. Review.

PMID:
15605385
[PubMed - indexed for MEDLINE]
15.

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]
16.

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]
Free Article
17.

Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation.

Molven A, Matre GE, Duran M, Wanders RJ, Rishaug U, Njølstad PR, Jellum E, Søvik O.

Diabetes. 2004 Jan;53(1):221-7.

PMID:
14693719
[PubMed - indexed for MEDLINE]
Free Article
18.

Regulation of leucine-stimulated insulin secretion and glutamine metabolism in isolated rat islets.

Li C, Najafi H, Daikhin Y, Nissim IB, Collins HW, Yudkoff M, Matschinsky FM, Stanley CA.

J Biol Chem. 2003 Jan 31;278(5):2853-8. Epub 2002 Nov 19.

PMID:
12444083
[PubMed - indexed for MEDLINE]
Free Article
19.

Acute insulin responses to leucine in children with the hyperinsulinism/hyperammonemia syndrome.

Kelly A, Ng D, Ferry RJ Jr, Grimberg A, Koo-McCoy S, Thornton PS, Stanley CA.

J Clin Endocrinol Metab. 2001 Aug;86(8):3724-8.

PMID:
11502802
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion.

Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE.

J Clin Invest. 2001 Aug;108(3):457-65.

PMID:
11489939
[PubMed - indexed for MEDLINE]
Free PMC Article

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