Encyclopedia of Parasitology

Living Edition
| Editors: Heinz Mehlhorn

Sickle Cell Disease

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27769-6_4325-1

In patients with sickle cell disease (endemic in sub-Saharan Africa and in regions of the Middle East) the hemoglobin (HbH) is alterated to HbS by mutation, so that the β-globin at position 6 glutamate is replaced by valine. Therefore the heterozygous carrier (HbAS) has a tenfold lower risk to be killed by a malaria infection during childhood (Aidoo et al. 2002) and as a consequence the allele is maintained at a frequency of 5–30 % in respective malaria endemic regions. However, until today the molecular mechanisms of the protection by the sickle cell hemoglobin (HbS) are still poorly understood (Cyrklaff et al. 2012). As hypothesis was established that hemoglobin S is unstable and open to oxidation of its iron from a ferrous (Fe2+) to a ferric (Fe3+) and subsequently to a ferryl (Fe4+) station. Since oxidized hemoglobin potentially prevents host actin remodeling, which is done normally by P. falciparum-stages, the merozoites cannot develop to mature schizonts inside erythrocytes of...

Keywords

Molecular Mechanism Lower Risk Sickle Cell Disease Sickle Cell Middle East 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
This is a preview of subscription content, log in to check access.

References

  1. Aidoo M et al (2002) Protective effects of the sickle cell gene against malaria morbidity and mortality. Lancet 359:1311–1312CrossRefPubMedGoogle Scholar
  2. Cyrklaff M et al (2012) Host actin remodeling and protection from malaria by hemoglobinopathies. Trends Parasitol 28:479–485CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Institut für Zoomorphologie, Zellbiologie und ParasitologieHeinrich-Heine-UniversitätDüsseldorfGermany