G6PDH

G6PDH or Glucose-6-phosphate dehydrogenase is an enzyme that plays a significant role in the Pentose Phosphate Pathway, a metabolic pathway parallel to glycolysis. It is the first enzyme in the pathway and is crucial for the production of NADPH, a coenzyme that aids in the protection of cells from oxidative damage.

Function

G6PDH catalyzes the conversion of glucose-6-phosphate into 6-phosphoglucono-δ-lactone, a step that is accompanied by the reduction of NADP+ to NADPH. This reaction is the first and rate-limiting step of the Pentose Phosphate Pathway. The NADPH produced is used in various biosynthetic reactions and in the defense against oxidative stress.

Clinical Significance

Deficiency in G6PDH is the most common human enzyme defect and is an X-linked, recessive hereditary disease. G6PD Deficiency can lead to symptoms such as neonatal jaundice, acute hemolytic anemia, and chronic nonspherocytic hemolytic anemia. The severity of the disease varies, and it is particularly prevalent in populations living in or originating from malaria-endemic areas.

Structure

G6PDH is a homodimeric protein, with each monomer composed of 514 amino acids. The active site of the enzyme is located in the N-terminal domain, while the C-terminal domain is responsible for the binding of the coenzyme NADP+.

See Also

• Pentose Phosphate Pathway
• Glycolysis
• NADPH
• G6PD Deficiency

G6PDH Resources
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