- Glucose-6-phosphate dehydrogenase
__NOTOC__Glucose-6-phosphate dehydrogenase (G6PD) is a cytosolic
enzymein the pentose phosphate pathway(see image), a metabolic pathwaythat supplies reducing energy to cells (such as erythrocytes) by maintaining the level of the co-enzymenicotinamide adenine dinucleotide phosphate (NADPH). The NADPH in turn maintains the level of glutathionein 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.In higher plants, several isoforms of G6PDH have been reported, which are localized in the cytosol, the plastidic stroma, and peroxisomes. [cite journal | first = | last = Corpas FJ et al. | title = A dehydrogenase-mediated recycling system of NADPH in plant peroxisomes | journal = Biochem. J.. | year = 1998 | volume = 330 | issue = 7 | pages = 777–784 | doi = ]
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.
glucose-6-phosphateinto 6-phosphoglucono-δ-lactoneand is the rate-limiting enzyme of the "pentose phosphate pathway". Glucose-6-phosphate dehydrogenase deficiencyis very common worldwide, and cause acute hemolytic anemiain 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 activityand associated clinical symptoms. Two transcript variants encoding different isoforms have been found for this gene. [cite web | title = Entrez Gene: G6PD glucose-6-phosphate dehydrogenase| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2539| accessdate = ]
Glucose-6-phosphate dehydrogenase deficiency
*cite journal | author=Vulliamy T, Beutler E, Luzzatto L |title=Variants of glucose-6-phosphate dehydrogenase are due to missense mutations spread throughout the coding region of the gene. |journal=Hum. Mutat. |volume=2 |issue= 3 |pages= 159–67 |year= 1993 |pmid= 8364584 |doi= 10.1002/humu.1380020302
*cite journal | author=Mason PJ |title=New insights into G6PD deficiency. |journal=Br. J. Haematol. |volume=94 |issue= 4 |pages= 585–91 |year= 1996 |pmid= 8826878 |doi=
*cite journal | author=Wajcman H, Galactéros F |title= [Glucose 6-phosphate dehydrogenase deficiency: a protection against malaria and a risk for hemolytic accidents] |journal=C. R. Biol. |volume=327 |issue= 8 |pages= 711–20 |year= 2004 |pmid= 15506519 |doi=
* [http://www.atsdr.cdc.gov/csem/nitrate/no3treatment_management.html#g6pd ATSDR - G6PD Deficiency]
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