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Items: 1 to 20 of 21

1.

Deletion of the gene encoding the ubiquitously expressed glucose-6-phosphatase catalytic subunit-related protein (UGRP)/glucose-6-phosphatase catalytic subunit-beta results in lowered plasma cholesterol and elevated glucagon.

Wang Y, Oeser JK, Yang C, Sarkar S, Hackl SI, Hasty AH, McGuinness OP, Paradee W, Hutton JC, Powell DR, O'Brien RM.

J Biol Chem. 2006 Dec 29;281(52):39982-9. Epub 2006 Oct 5.

2.

The molecular basis of type 1 glycogen storage diseases.

Chou JY.

Curr Mol Med. 2001 Mar;1(1):25-44. Review.

PMID:
11899241
3.

Type I glycogen storage diseases: disorders of the glucose-6-phosphatase/glucose-6-phosphate transporter complexes.

Chou JY, Jun HS, Mansfield BC.

J Inherit Metab Dis. 2015 May;38(3):511-9. doi: 10.1007/s10545-014-9772-x. Epub 2014 Oct 7. Review.

PMID:
25288127
4.

Adenovirus-mediated gene therapy in a mouse model of glycogen storage disease type 1a.

Chou JY, Zingone A, Pan CJ.

Eur J Pediatr. 2002 Oct;161 Suppl 1:S56-61. Epub 2002 Jul 19. Review.

PMID:
12373573
5.

Molecular genetics of type 1 glycogen storage disease.

Janecke AR, Mayatepek E, Utermann G.

Mol Genet Metab. 2001 Jun;73(2):117-25. Review.

PMID:
11386847
6.

Type I glycogen storage diseases: disorders of the glucose-6-phosphatase complex.

Chou JY, Matern D, Mansfield BC, Chen YT.

Curr Mol Med. 2002 Mar;2(2):121-43. Review.

PMID:
11949931
7.

The biochemistry and molecular biology of the glucose-6-phosphatase system.

Foster JD, Nordlie RC.

Exp Biol Med (Maywood). 2002 Sep;227(8):601-8. Review.

PMID:
12192101
8.

Glycogen storage disease type I and G6Pase-β deficiency: etiology and therapy.

Chou JY, Jun HS, Mansfield BC.

Nat Rev Endocrinol. 2010 Dec;6(12):676-88. doi: 10.1038/nrendo.2010.189. Epub 2010 Oct 26. Review.

9.

Historical highlights and unsolved problems in glycogen storage disease type 1.

Moses SW.

Eur J Pediatr. 2002 Oct;161 Suppl 1:S2-9. Epub 2002 Aug 23. Review.

PMID:
12373565
10.

Mutations in the glucose-6-phosphatase-alpha (G6PC) gene that cause type Ia glycogen storage disease.

Chou JY, Mansfield BC.

Hum Mutat. 2008 Jul;29(7):921-30. doi: 10.1002/humu.20772. Review.

11.

Glucose-6-phosphatase deficiency.

Froissart R, Piraud M, Boudjemline AM, Vianey-Saban C, Petit F, Hubert-Buron A, Eberschweiler PT, Gajdos V, Labrune P.

Orphanet J Rare Dis. 2011 May 20;6:27. doi: 10.1186/1750-1172-6-27. Review.

12.

New lessons in the regulation of glucose metabolism taught by the glucose 6-phosphatase system.

van de Werve G, Lange A, Newgard C, Méchin MC, Li Y, Berteloot A.

Eur J Biochem. 2000 Mar;267(6):1533-49. Review.

13.

Multiple roles of glucose-6-phosphatases in pathophysiology: state of the art and future trends.

Marcolongo P, Fulceri R, Gamberucci A, Czegle I, Banhegyi G, Benedetti A.

Biochim Biophys Acta. 2013 Mar;1830(3):2608-18. Review.

PMID:
23266497
14.

The SLC37 family of sugar-phosphate/phosphate exchangers.

Chou JY, Mansfield BC.

Curr Top Membr. 2014;73:357-82. doi: 10.1016/B978-0-12-800223-0.00010-4. Review.

15.

Gene therapy for type I glycogen storage diseases.

Chou JY, Mansfield BC.

Curr Gene Ther. 2007 Apr;7(2):79-88. Review.

16.

Neutropenia in type Ib glycogen storage disease.

Chou JY, Jun HS, Mansfield BC.

Curr Opin Hematol. 2010 Jan;17(1):36-42. doi: 10.1097/MOH.0b013e328331df85. Review.

17.

The SLC37 family of phosphate-linked sugar phosphate antiporters.

Chou JY, Sik Jun H, Mansfield BC.

Mol Aspects Med. 2013 Apr-Jun;34(2-3):601-11. doi: 10.1016/j.mam.2012.05.010. Review.

18.

Glucose-6-phosphatase catalytic subunit gene family.

Hutton JC, O'Brien RM.

J Biol Chem. 2009 Oct 23;284(43):29241-5. doi: 10.1074/jbc.R109.025544. Epub 2009 Aug 20. Review.

19.

The glucose-6-phosphatase system.

van Schaftingen E, Gerin I.

Biochem J. 2002 Mar 15;362(Pt 3):513-32. Review.

20.

Lessons from new mouse models of glycogen storage disease type 1a in relation to the time course and organ specificity of the disease.

Rajas F, Clar J, Gautier-Stein A, Mithieux G.

J Inherit Metab Dis. 2015 May;38(3):521-7. doi: 10.1007/s10545-014-9761-0. Epub 2014 Aug 28. Review.

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