Encyclopedia of Parasitology

2016 Edition
| Editors: Heinz Mehlhorn

Sickle Cell Disease

  • Heinz MehlhornEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-662-43978-4_4325

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...

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

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© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

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