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Glucose-6-phosphate dehydrogenase (G6PD) is an enzyme in the pentose phosphate pathway (see image, also known as the HMP shunt pathway). G6PD converts glucose-6-phosphate into 6-phosphoglucono-δ-lactone and is the rate-limiting enzyme of this metabolic pathway that supplies reducing energy to cells by maintaining the level of the co-enzyme nicotinamide adenine dinucleotide phosphate (NADPH). The NADPH in turn maintains the supply of reduced glutathione in the cells that is used to mop up free radicals that cause oxidative damage.
Glucose 6 Phophate Dehydrogenease is an enzyme that plays a key role in the pentose phosphate pathway [1]
Glucose-6-Phosphate Dehydrogenase Deficiency
Classification
The World Health Organization classifies G6PD genetic variants into five classes, the first three of which are deficiency states.[2]
- Severe deficiency (<10% activity) with chronic (nonspherocytic) hemolytic anemia
- Severe deficiency (<10% activity), with intermittent hemolysis
- Mild deficiency (10-60% activity), hemolysis with stressors only
- Non-deficient variant, no clinical sequelae
- Increased enzyme activity, no clinical sequelae
