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G6PD
G6PD Full Name
glucose-6-phosphate dehydrogenase
G6PD Introduction
Glucose-6-phosphate dehydrogenase (G6PD) is a crucial "housekeeping enzyme" found in various cells of living organisms. Its core physiological function is to catalyze the first and rate-limiting step of the pentose phosphate pathway (PPP). In this key reaction, G6PD oxidizes glucose-6-phosphate (G6P) and simultaneously reduces nicotinamide adenine dinucleotide phosphate (NADP+) to its reduced form, NADPH. For most cells, the PPP is the primary—or even sole—source of NADPH generation. NADPH serves as a vital cellular reducing power with profound physiological implications, particularly in maintaining cellular redox homeostasis. It provides essential electrons to glutathione reductase, ensuring the continuous regeneration of reduced glutathione (GSH). GSH is one of the most important intracellular antioxidants, capable of neutralizing and clearing reactive oxygen species (ROS)—such as hydrogen peroxide—generated by endogenous metabolism or external factors like drugs and infections, via the action of glutathione peroxidase. Thus, the proper functioning of G6PD is fundamental to protecting cells, especially red blood cells that are highly sensitive to oxidative stress, from oxidative damage.
Figure 1. Schematic diagram of G6PD dimer. (Source: Meng Q, et al. 2022)
Red blood cells, which lack a nucleus and mitochondria and cannot synthesize new proteins to replace damaged enzymes, rely almost entirely on glycolysis and the PPP for energy metabolism and antioxidant defense. G6PD deficiency (G6PDD) is the most common inherited enzyme deficiency in humans, following an X-linked incomplete dominant inheritance pattern. The core pathophysiological mechanism lies in mutations of the G6PD gene, leading to an enzyme protein with structural instability or significantly reduced catalytic activity. This results in insufficient G6PD activity within cells, particularly in mature red blood cells. Under normal physiological conditions, individuals with reduced G6PD activity often show no obvious symptoms; the NADPH production in their red blood cells is sufficient to handle basal levels of oxidative stress. However, this fragile balance is disrupted when the body is exposed to specific oxidative triggers. In such situations, the surge in intracellular ROS far exceeds the compensatory capacity of the NADPH that can be produced by red blood cells with low G6PD activity. The depletion of NADPH prevents the effective regeneration of reduced glutathione, stripping red blood cells of their critical defense against oxidative damage. High concentrations of ROS directly attack red blood cell membrane proteins (leading to altered membrane fluidity and permeability), hemoglobin (causing its oxidative denaturation and the formation of Heinz bodies), and other intracellular components, ultimately resulting in both intravascular and extravascular hemolysis, clinically manifested as acute hemolytic anemia.
Alternate Names for G6PD
G6PD; glucose-6-phosphate dehydrogenase; G6PD1; glucose-6-phosphate 1-dehydrogenase;
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