Abstract
SCD is the family of recessively inherited disorders of hemoglobin (Hb) [1], which have developed in response to strong evolutionary selection by malaria [2, 3]. Sickle-cell anemia (SCA), the most severe form of SCD, refers specifically to homozygosity for the HbS (βS), a variant of the HbB gene (which encodes β-globin), whereas people who inherit only one sickle gene are sickle-cell carriers [4, 5]. HbS homozygotes suffer from SCD, but heterozygotes have a tenfold reduced risk of severe malaria [6, 7]. SCD also includes other variants of the HbB gene, namely, HbC and HbE [8–11] and regulatory defects of HbA and HbB, which cause α and ß thalassemia (Sß + or Sß0) [12–14]. The evolutionary pressure has risen to high frequencies of HbS allele in malaria-exposed populations despite the fatal consequences for homozygotes (HbSS) [2]. Different populations have developed independent evolutionary responses to malaria at both the global and the local levels [15]. The most striking example is the Hb gene, in which three different coding single nucleotide points confer protection against malaria: Glu6Val (HbS), Glu6Lys (HbC), and Glu26Lys (HbE) [3]. The HbS allele is common in Africa but rare in Southeast Asia, whereas the opposite is true for the HbE allele [3, 15]. At the local level, not only frequency of the HbC and HbS allele varies [8], but the frequency of their haplotypes also varies; for instance, the HbS allele is found in four distinct haplotypes in West Africa region [16–18].
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Krejza, J., Arkuszewski, M., Swiat, M., Tomaszewski, M., Melhem, E.R. (2013). Sickle-Cell Disease and Stroke: Evidence-Based Neuroimaging. In: Medina, L.S., Sanelli, P.C., Jarvik, J.G. (eds) Evidence-Based Neuroimaging Diagnosis and Treatment. Evidence-Based Imaging. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-3320-0_12
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