Molecular basis and enzymatic properties of glucose 6-phosphate dehydrogenase volendam, leading to chronic nonspherocytic anemia, granulocyte dysfunction, and increased susceptibility to infections.

@article{Roos1999MolecularBA,
  title={Molecular basis and enzymatic properties of glucose 6-phosphate dehydrogenase volendam, leading to chronic nonspherocytic anemia, granulocyte dysfunction, and increased susceptibility to infections.},
  author={Dirk Roos and Rob van Zwieten and Juul Th. Wijnen and F{\'e}lix G{\'o}mez-Gallego and Martin de Boer and David Stevens and Claudia J Pronk-Admiraal and Theo de Rijk and C J F van Noorden and Ron S. Weening and Tom J. Vulliamy and J E Ploem and P. J. Mason and Jos{\'e} M Bautista and Pasha M. Khan and Ernest Beutler},
  journal={Blood},
  year={1999},
  volume={94 9},
  pages={2955-62}
}
We have investigated the blood cells from a woman with a low degree of chronic nonspherocytic hemolytic anemia and frequent bacterial infections accompanied by icterus and anemia. The activity of glucose 6-phosphate dehydrogenase (G6PD) in her red blood cells (RBCs) was below detection level, and in her leukocytes less than 3% of normal. In cultured skin fibroblasts, G6PD activity was approximately 15% of normal, with 4- to 5-fold increased Michaelis constant (Km) for NADP and for glucose 6… CONTINUE READING

Citations

Publications citing this paper.
Showing 1-10 of 39 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 41 references

The three - dimensional

  • P Rowland, AK Basak, S Gover, HR Levy, MJ Adams
  • Blood
  • 1996

Similar Papers

Loading similar papers…