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Gene Review

G6pdx  -  glucose-6-phosphate dehydrogenase X-linked

Mus musculus

Synonyms: G28A, G6PD, G6pd, G6pd-1, Glucose-6-phosphate 1-dehydrogenase X, ...
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Disease relevance of G6pdx

  • By analogy with the human X-chromosome, we conclude that the region in the mouse around the G6pd and St14-1 loci may contain two genes corresponding to distinct human myopathies: Emery Dreifuss muscular dystrophy which is known to be closely linked to St14-1 in man and the DMD homologue described here [1].
  • We report here that G6PD plays a role in adipogenesis and that its increase is tightly associated with the dysregulation of lipid metabolism and insulin resistance in obesity [2].
  • In human populations, many mutant G6PD alleles (some present at polymorphic frequencies) cause a partial loss of G6PD activity and a variety of hemolytic anemias, which vary from mild to severe [3].
  • Here we show that litters from untreated pregnant mutant mice with a hereditary G6PD deficiency had increased prenatal (fetal resorptions) and postnatal death [4].
  • G6PD deficiencies accordingly may have a broader biological relevance as important determinants of infertility, in utero and postnatal death, and teratogenesis [4].

High impact information on G6pdx

  • Inherited traits, such as abnormal hemoglobins and G-6-PD deficiency, and acquired cell-mediated immunity both subject malaria parasites to oxidant stress and may reinforce one another, increasing the chances of survival of children bearing these traits during the dangerous years of first exposure to malaria in areas where the disease is endemic [5].
  • To further our understanding of initiation and imprinting of X-chromosome inactivation, we have examined methylation of specific CpG sites of X-linked Pgk-1 and G6pd genes throughout female mouse development [6].
  • The minor subunit can be fully encoded by the X-linked G6PD cDNA, but the NH2-terminal region of the major subunit cannot [7].
  • We conclude that we have visualized the effects of a sporadic carcinogen induced somatic mutation in the G6PD gene of crypt stem cells and that a single stem cell maintains each colonic crypt [8].
  • We now show in female mice heterozygous for reduced expression of glucose-6-phosphate dehydrogenase (G6PD) activity that colonic epithelial cells express either normal or low enzyme activity, and form patches composed of multiple crypts of uniform phenotype [8].

Chemical compound and disease context of G6pdx


Biological context of G6pdx


Anatomical context of G6pdx

  • Consistently, G6PD knockdown via small interfering RNA attenuated adipocyte differentiation with less lipid droplet accumulation [2].
  • In 3T3-L1 cells, G6PD overexpression stimulated the expression of most adipocyte marker genes and elevated the levels of cellular free fatty acids, triglyceride, and FFA release [2].
  • Under the same conditions, G6PD tetramers were also found in extracts of spermatids and spermatozoa, indicating the presence of G6pd-2-encoded isoenzyme in these cell types [16].
  • Mouse chimeras from embryonic stem cells in which the X-linked glucose 6-phosphate dehydrogenase (G6PD) gene had been targeted were crossed with normal females [17].
  • By studying the in vitro differentiation of embryoid bodies produced from G6pdDelta ES cells that are totally unable to produce G6PD protein, we found that these cells are able to differentiate into mesodermal cells, cardiomyocytes, hepatocytes, and primitive erythroid cells [18].

Associations of G6pdx with chemical compounds


Other interactions of G6pdx

  • We have used X-chromosome inactivation in female embryos as a model system to study specific CpG sites in the X-linked Pgk-1 and G6pd housekeeping genes and in the imprinted regulatory Xist gene to elucidate the role of methylation in the initiation and maintenance of differential gene activity [21].
  • The data revealed that KIN-804 administration, followed or not by gamma irradiation, resulted in a significant decrease in GSH content in tumor tissues associated with inhibition in GR and G-6-PD activities [22].
  • Both catalase and G6PD were significantly increased in the RBCs of PKU animals [23].
  • The transcriptional organization of the region of the mouse X chromosome between the G6pd and the Fln1 genes was studied in detail, and it was compared with the syntenic region of the human chromosome [24].
  • This apoptotic death is delayed by reducing agents and by a caspase inhibitor, but it is prevented only by the restoration of G6PD activity [18].

Analytical, diagnostic and therapeutic context of G6pdx

  • We have isolated numerous clones, shown to be recombinant by Southern blot analysis, in which G6PD activity is undetectable [3].
  • Human-mouse comparative sequence analysis of the NEMO gene reveals an alternative promoter within the neighboring G6PD gene [25].
  • Microdissection of single crypts, showing either normal or low G6PD activity by histochemistry was performed in mice treated with ethylnitrosourea (ENU), and the presence of point mutations sought by PCR and direct sequencing [26].
  • By Western blot we have found a marked decrease in the G6PD protein levels in the GPDX mouse, with the C3H X GPDX heterozygote showing a lesser decrease [27].
  • In mice fed a low-fat diet, the relative amounts of G6PD mRNA were 3:1:1:0.38, respectively, in the four tissues [28].


  1. Localization of the region homologous to the Duchenne muscular dystrophy locus on the mouse X chromosome. Heilig, R., Lemaire, C., Mandel, J.L., Dandolo, L., Amar, L., Avner, P. Nature (1987) [Pubmed]
  2. Overexpression of glucose-6-phosphate dehydrogenase is associated with lipid dysregulation and insulin resistance in obesity. Park, J., Rho, H.K., Kim, K.H., Choe, S.S., Lee, Y.S., Kim, J.B. Mol. Cell. Biol. (2005) [Pubmed]
  3. Targeted disruption of the housekeeping gene encoding glucose 6-phosphate dehydrogenase (G6PD): G6PD is dispensable for pentose synthesis but essential for defense against oxidative stress. Pandolfi, P.P., Sonati, F., Rivi, R., Mason, P., Grosveld, F., Luzzatto, L. EMBO J. (1995) [Pubmed]
  4. An embryoprotective role for glucose-6-phosphate dehydrogenase in developmental oxidative stress and chemical teratogenesis. Nicol, C.J., Zielenski, J., Tsui, L.C., Wells, P.G. FASEB J. (2000) [Pubmed]
  5. The role of cell-mediated immune responses in resistance to malaria, with special reference to oxidant stress. Allison, A.C., Eugui, E.M. Annu. Rev. Immunol. (1983) [Pubmed]
  6. Methylation of CpG sites of two X-linked genes coincides with X-inactivation in the female mouse embryo but not in the germ line. Grant, M., Zuccotti, M., Monk, M. Nat. Genet. (1992) [Pubmed]
  7. Two structural genes on different chromosomes are required for encoding the major subunit of human red cell glucose-6-phosphate dehydrogenase. Kanno, H., Huang, I.Y., Kan, Y.W., Yoshida, A. Cell (1989) [Pubmed]
  8. Demonstration of somatic mutation and colonic crypt clonality by X-linked enzyme histochemistry. Griffiths, D.F., Davies, S.J., Williams, D., Williams, G.T., Williams, E.D. Nature (1988) [Pubmed]
  9. The localization of G6pd, glucose-6-phosphate dehydrogenase, and mdx, muscular dystrophy in the mouse X chromosome. Peters, J., Ball, S.T., Charles, D.J., Pretsch, W., Bulfield, G., Miller, D., Chapman, V.M. Genet. Res. (1988) [Pubmed]
  10. Glucose-6-phosphate dehydrogenase deficiency and the inflammatory response to endotoxin and polymicrobial sepsis. Wilmanski, J., Villanueva, E., Deitch, E.A., Spolarics, Z. Crit. Care Med. (2007) [Pubmed]
  11. Activity of divicine in Plasmodium vinckei-infected mice has implications for treatment of favism and epidemiology of G-6-PD deficiency. Clark, I.A., Cowden, W.B., Hunt, N.H., Maxwell, L.E., Mackie, E.J. Br. J. Haematol. (1984) [Pubmed]
  12. Glucose-6 phosphate dehydrogenase deficiency decreases the vascular response to angiotensin II. Matsui, R., Xu, S., Maitland, K.A., Hayes, A., Leopold, J.A., Handy, D.E., Loscalzo, J., Cohen, R.A. Circulation (2005) [Pubmed]
  13. Pulse-field linkage of the P3, G6pd and Cf-8 genes on the mouse X chromosome: demonstration of synteny at the physical level. Brockdorff, N., Amar, L.C., Brown, S.D. Nucleic Acids Res. (1989) [Pubmed]
  14. A 2.3-Mb yeast artificial chromosome contig spanning from Gabra3 to G6pd on the mouse X chromosome. Chatterjee, A., Faust, C.J., Molinari-Storey, L., Kiochis, P., Poustka, A., Herman, G.E. Genomics (1994) [Pubmed]
  15. Localization of the mouse Mcf-2 (Dbl) protooncogene within a conserved linkage group on the mouse X chromosome. Grant, S.G., Mattei, M.G., Galland, F., Stephenson, D.A., Keitz, B.T., Birnbaum, D., Chapman, V.M. Cytogenet. Cell Genet. (1990) [Pubmed]
  16. Testis-specific expression of a functional retroposon encoding glucose-6-phosphate dehydrogenase in the mouse. Hendriksen, P.J., Hoogerbrugge, J.W., Baarends, W.M., de Boer, P., Vreeburg, J.T., Vos, E.A., van der Lende, T., Grootegoed, J.A. Genomics (1997) [Pubmed]
  17. Maternally transmitted severe glucose 6-phosphate dehydrogenase deficiency is an embryonic lethal. Longo, L., Vanegas, O.C., Patel, M., Rosti, V., Li, H., Waka, J., Merghoub, T., Pandolfi, P.P., Notaro, R., Manova, K., Luzzatto, L. EMBO J. (2002) [Pubmed]
  18. G6PD is indispensable for erythropoiesis after the embryonic-adult hemoglobin switch. Paglialunga, F., Fico, A., Iaccarino, I., Notaro, R., Luzzatto, L., Martini, G., Filosa, S. Blood (2004) [Pubmed]
  19. Glucose-6-phosphate dehydrogenase plays a crucial role in protection from redox-stress-induced apoptosis. Fico, A., Paglialunga, F., Cigliano, L., Abrescia, P., Verde, P., Martini, G., Iaccarino, I., Filosa, S. Cell Death Differ. (2004) [Pubmed]
  20. Cytosolic NADP(+)-dependent isocitrate dehydrogenase status modulates oxidative damage to cells. Lee, S.M., Koh, H.J., Park, D.C., Song, B.J., Huh, T.L., Park, J.W. Free Radic. Biol. Med. (2002) [Pubmed]
  21. Epigenetic programming of differential gene expression in development and evolution. Monk, M. Dev. Genet. (1995) [Pubmed]
  22. Studies of methyl 2-nitroimidazole-1-acetohydroxamate (KIN-804) 1: effect on free radical scavenging system in mice bearing Ehrlich ascites carcinoma. Abu-Zeid, M., Hori, H., Nagasawa, H., Uto, Y., Inayama, S. Biol. Pharm. Bull. (2000) [Pubmed]
  23. Oxidative stress in a phenylketonuria animal model. Ercal, N., Aykin-Burns, N., Gurer-Orhan, H., McDonald, J.D. Free Radic. Biol. Med. (2002) [Pubmed]
  24. A comparative transcriptional map of a region of 250 kb on the human and mouse X chromosome between the G6PD and the FLN1 genes. Rivella, S., Tamanini, F., Bione, S., Mancini, M., Herman, G., Chatterjee, A., Maestrini, E., Toniolo, D. Genomics (1995) [Pubmed]
  25. Human-mouse comparative sequence analysis of the NEMO gene reveals an alternative promoter within the neighboring G6PD gene. Galgóczy, P., Rosenthal, A., Platzer, M. Gene (2001) [Pubmed]
  26. Somatic mutation of the glucose-6-phosphate dehydrogenase (g6pd) gene in colonic stem cells and crypt restricted loss of G6PD activity. Kuraguchi, M., Thomas, G.A., Williams, E.D. Mutat. Res. (1997) [Pubmed]
  27. A glucose-6-phosphate dehydrogenase (G6PD) splice site consensus sequence mutation associated with G6PD enzyme deficiency. Sanders, S., Smith, D.P., Thomas, G.A., Williams, E.D. Mutat. Res. (1997) [Pubmed]
  28. Structural characterization and tissue-specific expression of the mouse glucose-6-phosphate dehydrogenase gene. Hodge, D.L., Charron, T., Stabile, L.P., Klautky, S.A., Salati, L.M. DNA Cell Biol. (1998) [Pubmed]
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