Abstract

Sickle cell disease (SCD) is a genetic disease of hemoglobin (Hb) that occurs due to a non- conservative substitution of a polar glutamate (Glu) by non-polar valine (Val) in an invariant region, of hemoglobin β chain-subunit. This change distorts the normal Hb folding resulting in a sticky patch on the surface of the β-chains and associated complications. Nigeria has the largest burden of SCD globally with an estimated 150,000 new born affected annually. This review is aimed at analyzing the phytomedicines use in Nigeria to ameliorate the crisis associated with SCD and present probable target genetic loci/pathways that this phytomedicines could act upon for effective and enhanced management of SCD in a resource poor environment. Nucleation is essential in polymerization of Hb and phyto-medicine has been reported to inhibit this pathway. Different loci of genetic variation identified as modulator of SCD phenotype include nucleotide motifs within the β- globin gene cluster and distal genes located on different chromosomes. Fetal hemoglobin (HbF) is equally an important variable and modulator of the clinical features of SCD. Phytomedicine used in the management of SCD ameliorates oxidative stress and modulates cytokines that contributes to SCD complications. Here, we propose the use of phyto-medicine as key component for genetic modification, by twinkling, some transcription effectors/gene modulator such as B-cell lymphoma/leukemia 11A (BCL11A), or an erythroid specific transcription factor (KLF1), which are part of factors mediating HbF gene silencing.

Highlights

  • Sickle cell disease (SCD) occurs due to a non- conservative substitution of a polar glutamate (Glu) by non-polar valine (Val) in an invariant region, the sixth position of Hb-β chain-subunit (GAG/GTG; Glu (E) [6] Val (V); rs334) [5,6,7]

  • These extracts are thought to participate in biochemical processes and actions that include: activating metabolizing enzymes involved in carcinogenic detoxification; inhibiting reactive oxygen formation; blocking DNA adduct formation and in cell cycle regulation leading to apoptosis [32,53,76,77]

  • Moving forward, it is safe to argue, that the use of these plants with medicinal value in the management of SCD and other related ailments would continue to be a major part of the health care delivery system in Nigeria and many parts of the world

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Summary

Background

Sickle cell disease belongs to a family of disease known as hemoglobinopathies. In Nigeria, records showed that the three main tribes called the SCD by different names. The Yoruba’s called SCD sufferers “abiku” [1], the Ibos called it “Ogbanje” [1,2] and the Hausas called it “Sankara-miji” [1,3]. It is the commonest genetic disorder in Nigeria affecting about 4 million Nigerians at prevalence of 2% at birth while over 40 million individuals have sickle cell trait. We propose holistic inclusions of phytomedicines in SCD management

Introduction
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Future Perspectives and Conclusions
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