title: Glucose-6-Phosphate Dehydrogenase Deficiency and the Need for a Novel Treatment to Prevent Kernicterus.
abstract: Hyperbilirubinemia occurs frequently in newborns, and in severe cases can progress to kernicterus and permanent developmental disorders. Glucose-6-phosphate dehydrogenase (G6PD) deficiency, one of the most common human enzymopathies, is a major risk factor for hyperbilirubinemia and greatly increases the risk of kernicterus even in the developed world. Therefore, a novel treatment for kernicterus is needed, especially for G6PD-deficient newborns. Oxidative stress is a hallmark of bilirubin toxicity in the brain. We propose that the activation of G6PD via a small molecule chaperone is a potential strategy to increase endogenous defense against bilirubin-induced oxidative stress and prevent kernicterus.
authors.affiliation: Department of Chemical and Systems Biology, Stanford University, 269 Campus Drive, Stanford, CA 94305, USA.
authors.forename: Anna D
authors.initials: AD
authors.lastname: Cunningham
authors.affiliation: Department of Chemical and Systems Biology, Stanford University, 269 Campus Drive, Stanford, CA 94305, USA.
authors.forename: Sunhee
authors.initials: S
authors.lastname: Hwang
authors.affiliation: Department of Chemical and Systems Biology, Stanford University, 269 Campus Drive, Stanford, CA 94305, USA. Electronic address: mochly@stanford.edu.
authors.forename: Daria
authors.initials: D
authors.lastname: Mochly-Rosen
mesh_terms: D000975:Antioxidants / Q000627:therapeutic use*; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications / Q000378:metabolism*; D006801:Humans; D051556:Hyperbilirubinemia, Neonatal / Q000150:complications / Q000378:metabolism / Q000628:therapy*; D007231:Infant, Newborn; D007647:Kernicterus / Q000209:etiology / Q000378:metabolism / Q000517:prevention & control* / Q000628:therapy; D018832:Molecular Chaperones / Q000627:therapeutic use*; D010789:Phototherapy
chemical_list: D000975:Antioxidants; D018832:Molecular Chaperones
keywords: Activator; Bilirubin; Chaperone; G6PD deficiency; Hyperbilirubinemia; Jaundice; Kernicterus; Neurotoxicity