Glucose-6-phosphate dehydrogenase
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| Glucose-6-phosphate dehydrogenase
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| PDB rendering based on 1qki. | ||||||||||||||
| Available structures: 1qki, 2bh9, 2bhl | ||||||||||||||
| Identifiers | ||||||||||||||
| Symbol(s) | G6PD; G6PD1 | |||||||||||||
| External IDs | OMIM: 305900 MGI: 105979 Homologene: 37906 | |||||||||||||
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| RNA expression pattern | ||||||||||||||
| Orthologs | ||||||||||||||
| Human | Mouse | |||||||||||||
| Entrez | 2539 | 14381 | ||||||||||||
| Ensembl | ENSG00000160211 | ENSMUSG00000031400 | ||||||||||||
| Uniprot | P11413 | Q3TNL1 | ||||||||||||
| Refseq | NM_000402 (mRNA) NP_000393 (protein) | NM_008062 (mRNA) NP_032088 (protein) | ||||||||||||
| Location | Chr X: 153.41 - 153.43 Mb | Chr X: 70.66 - 70.68 Mb | ||||||||||||
| Pubmed search | [1] | [2] | ||||||||||||
Glucose-6-phosphate dehydrogenase (G6PD) is a cytosolic enzyme in the pentose phosphate pathway (see image), a metabolic pathway that supplies reducing energy to cells (such as erythrocytes) by maintaining the level of the co-enzyme nicotinamide adenine dinucleotide phosphate (NADPH). The NADPH in turn maintains the level of glutathione in these cells that helps protect the red blood cells against oxidative damage. Of greater quantitative importance is the production of NADPH for tissues actively engaged in biosynthesis of fatty acids and/or isoprenoids, such as the liver, mammary glands, adipose tissue, and the adrenal glands.
Regulation
Glucose-6-phosphate dehydrogenase is stimulated by its substrate NADP+. The usual ratio of NADPH/NADP+ in the cytosol of tissues engaged in biosyntheses is about 100/1. Increased utilization of NADPH for fatty acid biosynthesis will dramatically increase the level of NADP+, thus stimulating G6PD to produce more NADPH.
G6PD converts glucose-6-phosphate into 6-phosphoglucono-δ-lactone and is the rate-limiting enzyme of the pentose phosphate pathway.
Glucose-6-phosphate dehydrogenase deficiency is very common worldwide, and cause acute hemolytic anemia in the presence of simple infection, ingestion of fava beans, or reaction with certain medicines, AAA, antibiotics, antipyretics, and antimalarials.
G6PD is remarkable for its genetic diversity. Many variants of G6PD, mostly produced from missense mutations, have been described with wide ranging levels of enzyme activity and associated clinical symptoms. Two transcript variants encoding different isoforms have been found for this gene.[1]
See also
Further reading
- Vulliamy T, Beutler E, Luzzatto L (1993). "Variants of glucose-6-phosphate dehydrogenase are due to missense mutations spread throughout the coding region of the gene.". Hum. Mutat. 2 (3): 159-67. doi:10.1002/humu.1380020302. PMID 8364584.
- Mason PJ (1996). "New insights into G6PD deficiency.". Br. J. Haematol. 94 (4): 585-91. PMID 8826878.
- Wajcman H, Galactéros F (2004). "[Glucose 6-phosphate dehydrogenase deficiency: a protection against malaria and a risk for hemolytic accidents]". C. R. Biol. 327 (8): 711-20. PMID 15506519.
References
- IUBMB entry for 1.1.1.49
- BRENDA references for 1.1.1.49 (Recommended.)
- PubMed references for 1.1.1.49
- PubMed Central references for 1.1.1.49
- Google Scholar references for 1.1.1.49
External links
Oxidoreductases: alcohol oxidoreductases (EC 1.1) | |
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| 1.1.1 NAD/NADP acceptor | Carbohydrate dehydrogenases - Alcohol dehydrogenase - Glycerol-3-phosphate dehydrogenase - L-xylulose reductase - Aldose reductase - Lactate dehydrogenase - 3-Hydroxyacyl CoA dehydrogenase - Malate dehydrogenase - Isocitrate dehydrogenase - Phosphogluconate dehydrogenase - Glucose-6-phosphate dehydrogenase - HMG-CoA reductase - Β-Ketoacyl ACP reductase - Hydroxysteroid dehydrogenase: 11 Beta (HSD11B1, HSD11B2) - 3 Beta (3-beta-HSD) - 17 Beta - Carnitine dehydrogenase - Beta-hydroxybutyryl-CoA dehydrogenase - IMP dehydrogenase - DXP reductoisomerase |
| 1.1.3 oxygen acceptor | Glucose oxidase - L-gulonolactone oxidase - Xanthine oxidase |
Metabolism: carbohydrate metabolism - pentose phosphate pathway enzymes | |
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| oxidative | Glucose-6-phosphate dehydrogenase - 6-phosphogluconolactonase - Phosphogluconate dehydrogenase |
| nonoxidative | Phosphopentose isomerase - Phosphopentose epimerase - Transketolase - Transaldolase |
| see also disorders | |
de:Glucose-6-phosphat-Dehydrogenasehr:Glukoza-6-fosfat dehidrogenaza it:Glucosio-6-fosfato deidrogenasi ms:G6PD
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .

