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Gene Review

H6PD  -  hexose-6-phosphate dehydrogenase (glucose...

Homo sapiens

Synonyms: CORTRD1, G6PDH, GDH, GDH/6PGL endoplasmic bifunctional protein
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Disease relevance of H6PD

  • Glucose 6 phosphate dehydrogenase (G6PDH) deficiency is the most frequent cause of hemolytic anemias due to enzyme abnormality [1].
  • This study demonstrates that a mild degree of G-6-PDH deficiency (comparable to the human class III G-6-PDH deficiencies) worsens erythrocyte dysfunction during sepsis [2].
  • Evidence for functional convergence of redox regulation in G6PDH isoforms of cyanobacteria and higher plants [3].
  • Increased erythrocyte rigidity and tendency for hemolysis together with alterations in band 3-spectrin interactions may contribute to the immunomodulatory effects of G-6-PDH deficiency observed after major trauma and infections in humans [2].
  • It appeared that after 5 min of incubation at 37 degrees C the residual activity of G6PDH in an atmosphere of oxygen compared with nitrogen was 0% in normal liver tissue and 15% in normal colon epithelium, whereas in colon carcinoma and in colon carcinoma metastasis in liver it was 48% and 33%, respectively [4].

Psychiatry related information on H6PD


High impact information on H6PD

  • The dissociation is reversible, and there are no permanent modifications of either G6PDH or its particulate binding site that affect binding [7].
  • In unfertilized eggs of the sea urchin, Strongylocentrotus purpuratus, glucose-6-phosphate dehydrogenase (G6PDH) associates with the particulate elements remaining either after homogenization or extraction of eggs with non-ionic detergent in low ionic-strength media [7].
  • In contrast, the variation of IFN-alphaR2c/G6PDH ratio at diagnosis was significantly associated with the achievement of major cytogenetic response (MCR; 34% or lower Ph(+) metaphases) [8].
  • The enzyme, glucose-6-phosphate dehydrogenase (G6PDH, EC1.1.1.49), has long been considered and studied as the archetypical X-linked "housekeeping" enzyme that is present in all cells, where it plays the key role in regulating carbon flow through the pentose phosphate pathway [9].
  • The molecular chaperone GroEL reverses the refolding kinetics of G6PDH from biphase back to monophase and accelerates the refolding process [10].

Chemical compound and disease context of H6PD

  • We tested this hypothesis by demonstrating NAD-dependent lactate dehydrogenase (LDH) activity instead of NADP-dependent G6PDH activity in normal rat liver and colon, in human colon carcinoma, and in experimentally induced metastases of colon carcinoma in rat livers [4].
  • Products of the reaction of 4-hydroxy-2-nonenal (4HNE) with native and heat-denatured Leuconostoc mesenteroides glucose-6-phosphate dehydrogenase (G6PDH) were analyzed to determine the structure and position of the protein modifications [11].
  • We determined the activity of G-6-PDH as well as the penetration and metabolism of DHEA - diminished plasma concentrations of which have been found in psoriatics previously - in 16 patients with active psoriasis and 16 controls [12].
  • It is possible that the intensification of G6PDH activity in carcinomas is a sign of the shift of the carbohydrate metabolism from an aerobic path or that the activity of the pentose shunt is higher because of the increased need for nucleic acid precursors in tissues with faster growth rates [13].
  • NADPH is a powerful regulator of G6PDH activity in the normal astrocytes and in gliomas [14].

Biological context of H6PD

  • CONCLUSIONS: Digenic triallelic genotypes of the H6PD R453Q variant and HSD11B1 83557insA mutation do not always cause CRD [15].
  • On the contrary, the H6PD R453Q variant is associated with PCOS and might influence its phenotype by influencing adrenal activity [15].
  • Results: No influence of the HSD11B1 83,557insA (allele frequencies 22.0 and 21.5%) and H6PD R453Q (allele frequencies 22.9 and 20.2%) variants was found for the different outcome measures that were investigated, either separately or when at least three alleles were affected [5].
  • Novel SNPs were identified in both affected and unaffected individuals in GPR157 (c.795C>T [Arg218Leu]; c.811C>T [Ala223Val]), MGC4399 (c.1024G>C [Leu277Leu]), and H6PD (c.754A>C [Asp151Ala]) [16].
  • Osmotic change, phosphate sequestration, or oxidative stress did not affect cytosolic G6PDH activity [17].

Anatomical context of H6PD


Associations of H6PD with chemical compounds

  • Context: Recently, it was proposed that a combination of the 83,557insA polymorphism in the 11beta-hydroxysteroid dehydrogenase type 1 (HSD11B1) gene and the R453Q polymorphism in the hexose-6-phosphate dehydrogenase (H6PD) gene interacts to cause cortisone reductase deficiency (CRD) when at least three alleles are affected [5].
  • Using the published protein sequence from a rabbit microsomal glucose-6-phosphate dehydrogenase G6PD we have isolated and sequenced a cDNA clone coding for its human equivalent, which is also known as hexose-6-phosphate dehydrogenase (H6PD) and glucose dehydrogenase [21].
  • In all species except the goby, two groups of isozymes were distinguished, corresponding to the mammalian G6PD (specific for glucose-6-phosphate (G6P) and NADP+) and H6PD (capable of utilizing galactose-6-phosphate and in certain cases other monosaccharide phosphates in addition to G6P) [22].
  • The effects of polysaccharide, polyethylene glycol, and protein-crowding agents on the refolding of glucose-6-phosphate dehydrogenase (G6PDH) and protein disulfide isomerase have been examined [10].
  • Obviously, in extended dark periods, G6PDH activity in the stroma is restricted but can be stimulated in response to high demands for NADPH [17].

Other interactions of H6PD


Analytical, diagnostic and therapeutic context of H6PD


  1. Perioperative management of glucose 6 phosphate dehydrogenase deficiency. A review of the literature. Muñoz Corsini, L., Dominguez, E., Mourelle, I., Galindo, S., Porras, M.C. Minerva anestesiologica. (1999) [Pubmed]
  2. Red blood cell dysfunction in septic glucose-6-phosphate dehydrogenase-deficient mice. Spolarics, Z., Condon, M.R., Siddiqi, M., Machiedo, G.W., Deitch, E.A. Am. J. Physiol. Heart Circ. Physiol. (2004) [Pubmed]
  3. Evidence for functional convergence of redox regulation in G6PDH isoforms of cyanobacteria and higher plants. Wendt, U.K., Hauschild, R., Lange, C., Pietersma, M., Wenderoth, I., von Schaewen, A. Plant Mol. Biol. (1999) [Pubmed]
  4. The histochemical G6PDH reaction but not the LDH reaction with neotetrazolium is suitable for the oxygen sensitivity test to detect cancer cells. Griffini, P., Vigorelli, E., Jonges, G.N., Van Noorden, C.J. J. Histochem. Cytochem. (1994) [Pubmed]
  5. Lack of Association of the 11{beta}-Hydroxysteroid Dehydrogenase Type 1 Gene 83,557insA and Hexose-6-Phosphate Dehydrogenase Gene R453Q Polymorphisms with Body Composition, Adrenal Androgen Production, Blood Pressure, Glucose Metabolism, and Dementia. Smit, P., Dekker, M.J., de Jong, F.J., van den Beld, A.W., Koper, J.W., Pols, H.A., Brinkmann, A.O., de Jong, F.H., Breteler, M.M., Lamberts, S.W. J. Clin. Endocrinol. Metab. (2007) [Pubmed]
  6. Oxidative stress and antioxidant defenses after prolonged starvation in Dentex dentex liver. Morales, A.E., Pérez-Jiménez, A., Hidalgo, M.C., Abellán, E., Cardenete, G. Comp. Biochem. Physiol. C Toxicol. Pharmacol. (2004) [Pubmed]
  7. Regulation of glucose-6-phosphate dehydrogenase activity in sea urchin eggs by reversible association with cell structural elements. Swezey, R.R., Epel, D. J. Cell Biol. (1986) [Pubmed]
  8. Expression of interferon-alpha (IFN-alpha) receptor 2c at diagnosis is associated with cytogenetic response in IFN-alpha-treated chronic myeloid leukemia. Barthe, C., Mahon, F.X., Gharbi, M.J., Fabères, C., Bilhou-Nabéra, C., Hochhaus, A., Reiffers, J., Marit, G. Blood (2001) [Pubmed]
  9. Glucose-6-phosphate dehydrogenase: a "housekeeping" enzyme subject to tissue-specific regulation by hormones, nutrients, and oxidant stress. Kletzien, R.F., Harris, P.K., Foellmi, L.A. FASEB J. (1994) [Pubmed]
  10. Effects of macromolecular crowding on the refolding of glucose- 6-phosphate dehydrogenase and protein disulfide isomerase. Li, J., Zhang, S., Wang, C. J. Biol. Chem. (2001) [Pubmed]
  11. Determination of site-specific modifications of glucose-6-phosphate dehydrogenase by 4-hydroxy-2-nonenal using matrix assisted laser desorption time-of-flight mass spectrometry. Grace, J.M., MacDonald, T.L., Roberts, R.J., Kinter, M. Free Radic. Res. (1996) [Pubmed]
  12. Augmented glucose-6-phosphate dehydrogenase activity and normal penetration and metabolism of dehydroepiandrosterone in mononuclear leukocytes in psoriasis. Schopf, R.E., Müller, F.J., Benes, P., Morsches, B. Arch. Dermatol. Res. (1986) [Pubmed]
  13. Semiquantitative cytochemical estimation of glucose-6-phosphate dehydrogenase activity in benign diseases and carcinoma of the breast. Bokun, R., Bakotin, J., Milasinović, D. Acta Cytol. (1987) [Pubmed]
  14. Regulation of the pentose phosphate pathway in human astrocytes and gliomas. Loreck, D.J., Galarraga, J., Van der Feen, J., Phang, J.M., Smith, B.H., Cummins, C.J. Metabolic brain disease. (1987) [Pubmed]
  15. A study of the hexose-6-phosphate dehydrogenase gene R453Q and 11beta-hydroxysteroid dehydrogenase type 1 gene 83557insA polymorphisms in the polycystic ovary syndrome. San Millán, J.L., Botella-Carretero, J.I., Alvarez-Blasco, F., Luque-Ramírez, M., Sancho, J., Moghetti, P., Escobar-Morreale, H.F. J. Clin. Endocrinol. Metab. (2005) [Pubmed]
  16. Analysis of fifteen positional candidate genes for Schnyder crystalline corneal dystrophy. Aldave, A.J., Rayner, S.A., Principe, A.H., Affeldt, J.A., Katsev, D., Yellore, V.S. Mol. Vis. (2005) [Pubmed]
  17. Differential regulation of glucose-6-phosphate dehydrogenase isoenzyme activities in potato. Hauschild, R., von Schaewen, A. Plant Physiol. (2003) [Pubmed]
  18. Catalysis-electrochemical determination of zeptomole enzyme and its application for single-cell analysis. Sun, X., Jin, W. Anal. Chem. (2003) [Pubmed]
  19. A dehydrogenase-mediated recycling system of NADPH in plant peroxisomes. Corpas, F.J., Barroso, J.B., Sandalio, L.M., Distefano, S., Palma, J.M., Lupiáñez, J.A., Del Río, L.A. Biochem. J. (1998) [Pubmed]
  20. Dehydroepiandrosterone and 16 alpha-Br-epiandrosterone inhibit 12-O-tetradecanoylphorbol-13-acetate stimulation of superoxide radical production by human polymorphonuclear leukocytes. Whitcomb, J.M., Schwartz, A.G. Carcinogenesis (1985) [Pubmed]
  21. Human hexose-6-phosphate dehydrogenase (glucose 1-dehydrogenase) encoded at 1p36: coding sequence and expression. Mason, P.J., Stevens, D., Diez, A., Knight, S.W., Scopes, D.A., Vulliamy, T.J. Blood Cells Mol. Dis. (1999) [Pubmed]
  22. Glucose-6-phosphate dehydrogenase isozymes in fish--a comparative study. Kidder, G.M. J. Exp. Zool. (1983) [Pubmed]
  23. A new continuous automated assay for the determination of intestinal lactase. Sall, I., Férard, G. Digestion (2003) [Pubmed]
  24. Intersubunit disulfide interactions play a critical role in maintaining the thermostability of glucose-6-phosphate dehydrogenase from the hyperthermophilic bacterium Aquifex aeolicus. Nakka, M., Iyer, R.B., Bachas, L.G. Protein J. (2006) [Pubmed]
  25. Adaptational changes in kinetic parameters of G6PDH but not of PGDH during contamination-induced carcinogenesis in livers of North Sea flatfish. Van Noorden, C.J., Bahns, S., Köhler, A. Biochim. Biophys. Acta (1997) [Pubmed]
  26. Homogeneous enzyme immunoassay of acetaminophen in serum. Hepler, B., Weber, J., Sutheimer, C., Sunshine, I. Am. J. Clin. Pathol. (1984) [Pubmed]
  27. Effect of sucrose/raffinose mass ratios on the stability of co-lyophilized protein during storage above the Tg. Davidson, P., Sun, W.Q. Pharm. Res. (2001) [Pubmed]
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