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Glucose-6-Phosphate Dehydrogenase Deficiency and the Need for a Novel Treatment to Prevent Kernicterus

Anna D Cunningham1, Sunhee Hwang1, Daria Mochly-Rosen1

  • 1Department of Chemical and Systems Biology, Stanford University, 269 Campus Drive, Stanford, CA 94305, USA.

Clinics in Perinatology
|May 29, 2016
PubMed

Insights

Glucose-6-phosphate dehydrogenase (G6PD) deficiency increases newborn kernicterus risk. Activating G6PD with a chaperone may boost defenses against bilirubin-induced oxidative stress, preventing brain damage.

Area of Science:

  • Biochemistry
  • Neonatal Medicine
  • Neuroscience

Background:

  • Hyperbilirubinemia is common in newborns and can lead to kernicterus, causing permanent developmental issues.
  • Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a prevalent enzymopathy and a significant risk factor for severe hyperbilirubinemia and kernicterus.
  • Oxidative stress is a key mechanism in bilirubin-induced brain toxicity.

Purpose of the Study:

  • To propose a novel therapeutic strategy for preventing kernicterus, particularly in G6PD-deficient newborns.
  • To explore the potential of activating G6PD as a method to combat bilirubin toxicity.

Main Methods:

  • The study proposes a theoretical approach involving small molecule chaperones.
  • The proposed method aims to enhance the activity of the G6PD enzyme.

Main Results:

  • Activation of G6PD is hypothesized to increase endogenous antioxidant defenses.
  • This enhanced defense mechanism could mitigate bilirubin-induced oxidative stress in the brain.

Conclusions:

  • Small molecule chaperone-mediated G6PD activation presents a promising strategy for kernicterus prevention.
  • This approach offers a potential novel treatment for G6PD-deficient newborns at high risk for kernicterus.

Keywords:
ActivatorBilirubinChaperoneG6PD deficiencyHyperbilirubinemiaJaundiceKernicterusNeurotoxicity

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