Humanized mouse model of glucose 6-phosphate dehydrogenase deficiency for in vivo assessment of hemolytic toxicity
Abstract
This article describes the development and validation of a novel mouse model that can be used to predict hemolytic toxicity of drugs that occurs in individuals with an enzyme deficiency known as glucose-6-phosphate dehydrogenase (G6PD) deficiency. G6PD deficiency affects more than 400 million people worldwide. In this model, nonobese diabetic/SCID mice are transfused with human RBCs from G6PD-deficient donors. Treatment with drugs known to cause hemolytic anemia in humans do not cause damage to the mouse RBCs nor to the transfused normal human RBCs; but a robust hemolytic response is observed in the mice transfused with G6PD-deficient human RBCs. The immediate impact of this model will be in advancing the development antimalarial drugs.
- Publication:
-
Proceedings of the National Academy of Science
- Pub Date:
- October 2013
- DOI:
- 10.1073/pnas.1310402110
- Bibcode:
- 2013PNAS..11017486R