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Items: 1 to 20 of 110

1.

Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase.

Li C, Chen P, Palladino A, Narayan S, Russell LK, Sayed S, Xiong G, Chen J, Stokes D, Butt YM, Jones PM, Collins HW, Cohen NA, Cohen AS, Nissim I, Smith TJ, Strauss AW, Matschinsky FM, Bennett MJ, Stanley CA.

J Biol Chem. 2010 Oct 8;285(41):31806-18. doi: 10.1074/jbc.M110.123638. Epub 2010 Jul 29.

2.

Leucine-sensitive hyperinsulinaemic hypoglycaemia in patients with loss of function mutations in 3-Hydroxyacyl-CoA Dehydrogenase.

Heslegrave AJ, Kapoor RR, Eaton S, Chadefaux B, Akcay T, Simsek E, Flanagan SE, Ellard S, Hussain K.

Orphanet J Rare Dis. 2012 May 14;7:25. doi: 10.1186/1750-1172-7-25.

3.

Novel insights into fatty acid oxidation, amino acid metabolism, and insulin secretion from studying patients with loss of function mutations in 3-hydroxyacyl-CoA dehydrogenase.

Heslegrave AJ, Hussain K.

J Clin Endocrinol Metab. 2013 Feb;98(2):496-501. doi: 10.1210/jc.2012-3134. Epub 2012 Dec 18. Review.

PMID:
23253615
4.

Role of medium- and short-chain L-3-hydroxyacyl-CoA dehydrogenase in the regulation of body weight and thermogenesis.

Schulz N, Himmelbauer H, Rath M, van Weeghel M, Houten S, Kulik W, Suhre K, Scherneck S, Vogel H, Kluge R, Wiedmer P, Joost HG, Schürmann A.

Endocrinology. 2011 Dec;152(12):4641-51. doi: 10.1210/en.2011-1547. Epub 2011 Oct 11.

5.

Glutaminolysis and insulin secretion: from bedside to bench and back.

Kelly A, Li C, Gao Z, Stanley CA, Matschinsky FM.

Diabetes. 2002 Dec;51 Suppl 3:S421-6.

6.

Two genetic forms of hyperinsulinemic hypoglycemia caused by dysregulation of glutamate dehydrogenase.

Stanley CA.

Neurochem Int. 2011 Sep;59(4):465-72. doi: 10.1016/j.neuint.2010.11.017. Epub 2010 Dec 2. Review.

7.

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.

8.

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.

9.

Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose-fatty acid cycle?

Eaton S, Chatziandreou I, Krywawych S, Pen S, Clayton PT, Hussain K.

Biochem Soc Trans. 2003 Dec;31(Pt 6):1137-9. Review.

PMID:
14641012
10.

The Hypoglycemic Phenotype Is Islet Cell-Autonomous in Short-Chain Hydroxyacyl-CoA Dehydrogenase-Deficient Mice.

Molven A, Hollister-Lock J, Hu J, Martinez R, Njølstad PR, Liew CW, Weir G, Kulkarni RN.

Diabetes. 2016 Jun;65(6):1672-8. doi: 10.2337/db15-1475. Epub 2016 Mar 7.

11.

Delineation of glutamate pathways and secretory responses in pancreatic islets with β-cell-specific abrogation of the glutamate dehydrogenase.

Vetterli L, Carobbio S, Pournourmohammadi S, Martin-Del-Rio R, Skytt DM, Waagepetersen HS, Tamarit-Rodriguez J, Maechler P.

Mol Biol Cell. 2012 Oct;23(19):3851-62. doi: 10.1091/mbc.E11-08-0676. Epub 2012 Aug 8.

12.

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
13.

Insulin secretion profiles are modified by overexpression of glutamate dehydrogenase in pancreatic islets.

Carobbio S, Ishihara H, Fernandez-Pascual S, Bartley C, Martin-Del-Rio R, Maechler P.

Diabetologia. 2004 Feb;47(2):266-76. Epub 2003 Dec 20.

PMID:
14689183
14.

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. doi: 10.3945/ajcn.2009.27462AA. Epub 2009 Jul 22. Review.

16.

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.

17.

Mechanisms of amino acid-stimulated insulin secretion in congenital hyperinsulinism.

Zhang T, Li C.

Acta Biochim Biophys Sin (Shanghai). 2013 Jan;45(1):36-43. doi: 10.1093/abbs/gms107. Epub 2012 Dec 4. Review.

18.

Overexpression of constitutively activated glutamate dehydrogenase induces insulin secretion through enhanced glutamate oxidation.

Anno T, Uehara S, Katagiri H, Ohta Y, Ueda K, Mizuguchi H, Moriyama Y, Oka Y, Tanizawa Y.

Am J Physiol Endocrinol Metab. 2004 Feb;286(2):E280-5. Epub 2003 Oct 7.

19.

Mitochondrial GTP insensitivity contributes to hypoglycemia in hyperinsulinemia hyperammonemia by inhibiting glucagon release.

Kibbey RG, Choi CS, Lee HY, Cabrera O, Pongratz RL, Zhao X, Birkenfeld AL, Li C, Berggren PO, Stanley C, Shulman GI.

Diabetes. 2014 Dec;63(12):4218-29. doi: 10.2337/db14-0783. Epub 2014 Jul 14.

20.

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

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