Glucose-6-Phosphate Dehydrogenase Deficiency

Enzymes in the human body act as catalysts for important chemical reactions in cellular metabolism. As such, a deficiency of a particular enzyme can translate to a life-threatening disease. G6PD (glucose-6-phosphate dehydrogenase) deficiency, a genetic condition that results in a shortage of the enzyme glucose-6-phosphate dehydrogenase, is the most common enzyme deficiency in humans. This enzyme, shown in the figure below, is the rate-limiting enzyme for the metabolic pathway that supplies NADPH to cells (the figure below).

A colorful model of the Glucose-6-phosphate dehydrogenase structure is shown. The molecule has two distinct lobes which are filled with spiraled ribbon-like regions of yellow, lavender, blue, silver, green, and pink.

Glucose-6-phosphate dehydrogenase is a rate-limiting enzyme for the metabolic pathway that supplies NADPH to cells.

A disruption in this pathway can lead to reduced glutathione in red blood cells; once all glutathione is consumed, enzymes and other proteins such as hemoglobin are susceptible to damage. For example, hemoglobin can be metabolized to bilirubin, which leads to jaundice, a condition that can become severe. People who suffer from G6PD deficiency must avoid certain foods and medicines containing chemicals that can trigger damage their glutathione-deficient red blood cells.

A reaction mechanism is diagrammed in this figure. At the left, the name Glucose is followed by a horizontal, right pointing arrow, labeled, “Hexokinase.” Below this arrow and to the left is a yellow star shape labeled, “A T P.” A curved arrow extends from this shape to the right pointing arrow, and down to the right to a small brown oval labeled, “A D P.” To the right of the horizontal arrow is the name Glucose 6 phosphate, which is followed by another horizontal, right pointing arrow which is labeled, “G 6 P D.” A small orange rectangle below and left of this arrow is labeled “N A D P superscript plus.” A curved arrow extends from this shape to the right pointing arrow, and down to the right to a small salmon-colored rectangle labeled “N A P D H.” A curved arrow extends from this shape below and to the left, back to the orange rectangle labeled, “N A D P superscript plus.” Another curved arrow extends from a green oval labeled “G S S G” below the orange rectangle, up to the arrow curving back to the orange rectangle. This last curved arrow continues on to the lower right to a second green oval labeled, “G S H.” The end of this curved arrow is labeled, “Glutathione reductase.” To the right of the rightmost horizontal arrow appears the name 6 phosphogluconate.

In the mechanism for the pentose phosphate pathway, G6PD catalyzes the reaction that regulates NADPH, a co-enzyme that regulates glutathione, an antioxidant that protects red blood cells and other cells from oxidative damage.

This lesson is part of:

Chemical Kinetics

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