Glucose-6-phosphatase catalytic subunit gene family.
Glucose-6-phosphatase catalyzes the hydrolysis of glucose 6-phosphate (G6P) to glucose and inorganic phosphate. It is a multicomponent system located in the endoplasmic reticulum that comprises several integral membrane proteins, namely a catalytic subunit (G6PC) and transporters for G6P, inorganic phosphate, and glucose. The G6PC gene family contains three members, designated G6PC, G6PC2, and G6PC3. The tissue-specific expression patterns of these genes differ, and mutations in all three genes have been linked to distinct diseases in humans. This minireview discusses the disease association and transcriptional regulation of the G6PC genes as well as the biological functions of the encoded proteins.
Hutton
John C
JC
Barbara Davis Center for Childhood Diabetes, University of Colorado at Denver, Aurora, Colorado 80045, USA.
O'Brien
Richard M
RM
D000818:Animals; D002239:Carbohydrate Metabolism, Inborn Errors / Q000201:enzymology* / Q000235:genetics; D002352:Carrier Proteins / Q000235:genetics / Q000378:metabolism; D020134:Catalytic Domain / Q000235:genetics; D004721:Endoplasmic Reticulum / Q000201:enzymology* / Q000235:genetics; D015971:Gene Expression Regulation, Enzymologic; D005952:Glucose-6-Phosphatase / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D006868:Hydrolysis; D005810:Multigene Family; D046912:Multiprotein Complexes / Q000235:genetics / Q000378:metabolism; D009154:Mutation; D009928:Organ Specificity; D014158:Transcription, Genetic / Q000235:genetics
D002352:Carrier Proteins; D046912:Multiprotein Complexes; D005952:Glucose-6-Phosphatase
J Nutr. 2007 Mar;137(3):554-9
17311939
J Biol Chem. 1998 Jan 2;273(1):228-34
9417069
Hum Mutat. 2008 Jul;29(7):921-30
18449899
Eur J Endocrinol. 1997 Feb;136(2):137-45
9116905
J Biol Chem. 2003 Aug 29;278(35):32485-8
12788933
Am J Physiol Endocrinol Metab. 2007 Mar;292(3):E788-801
17106062
J Biol Chem. 2001 Jul 6;276(27):25197-207
11297555
J Clin Invest. 2006 Dec;116(12):3258-65
17143333
Proc Natl Acad Sci U S A. 2003 Jul 8;100(14):8384-8
12815107
J Immunol. 2006 Mar 1;176(5):2781-9
16493034
J Biol Chem. 1998 Mar 13;273(11):6144-8
9497333
Science. 1993 Oct 22;262(5133):580-3
8211187
Diabetes. 1999 Mar;48(3):543-51
10078554
Nat Genet. 1996 Jun;13(2):203-9
8640227
Nature. 2008 Nov 13;456(7219):269-73
18849969
J Mol Endocrinol. 2002 Oct;29(2):205-22
12370122
Diabetes. 1999 May;48(5):1198-214
10331429
Mol Endocrinol. 2006 Nov;20(11):2831-47
16840535
BMJ. 2001 Jan 6;322(7277):15-8
11141143
FASEB J. 2008 Jul;22(7):2206-13
18337460
J Biol Chem. 1993 Oct 15;268(29):21482-5
8407995
Curr Gene Ther. 2007 Apr;7(2):79-88
17430128
Science. 2008 Nov 28;322(5906):1395-9
19039140
J Mol Endocrinol. 2004 Feb;32(1):33-53
14765991
J Biol Chem. 2006 Dec 29;281(52):39982-9
17023421
Diabetologia. 2007 Apr;50(4):774-8
17265032
Diabetes. 2004 Jul;53(7):1754-64
15220199
Am J Physiol Endocrinol Metab. 2004 May;286(5):E795-808
14722027
Nature. 2006 Oct 5;443(7111):E10-1
17024043
Diabetes. 2001 Mar;50(3):502-14
11246869
Diabetes. 2008 Jan;57(1):133-41
17942825
Nature. 2003 May 29;423(6939):550-5
12754525
Proc Soc Exp Biol Med. 1997 Sep;215(4):314-32
9270716
J Clin Invest. 1988 Jun;81(6):1953-61
3290257
Biochem J. 2003 May 1;371(Pt 3):675-86
12540293
Diabetes. 1999 Mar;48(3):531-42
10078553
J Biol Chem. 2003 Nov 21;278(47):47098-103
13129915
J Biol Chem. 2002 Mar 29;277(13):10912-21
11782487
J Clin Invest. 2007 Mar;117(3):784-93
17318259
FEBS Lett. 2003 Sep 11;551(1-3):159-64
12965222
J Biol Chem. 2004 Aug 13;279(33):34277-89
15180990
Nat Med. 2005 Jun;11(6):645-52
15908957
Eur J Biochem. 2000 Mar;267(6):1533-49
10712583
Science. 2008 May 23;320(5879):1085-8
18451265
J Biol Chem. 2004 Apr 2;279(14):13976-83
14722102
N Engl J Med. 2009 Jan 1;360(1):32-43
19118303
FEBS Lett. 2004 Mar 26;562(1-3):160-4
15044018
J Biol Chem. 2004 Jun 18;279(25):26215-9
15087461
Biochem Soc Trans. 2001 Aug;29(Pt 4):552-8
11498027
Biochem J. 2002 Mar 15;362(Pt 3):513-32
11879177
Mol Endocrinol. 2005 Dec;19(12):3001-22
16037130
J Mol Endocrinol. 2008 Nov;41(5):315-28
18753309