Sickle Cell Disease (SCD)

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Sickle Cell Disease (SCD) is genetic disorder1, affecting 100,000 individuals in the United States2, resulting from a single amino acid substitution3, which causes abnormality to hemoglobin; a protein contained in red blood cells (RBCs) 4.
Sickle Cell Disease, characterized by chronic inflammation and enhanced release of hemoglobin (Hb) 5, is a hemolytic disease6. Hemolysis, the rupture of red blood cells7, can occur either intravascularly or extravascularly8 and tends to occur more frequently in SCD patients9. Intravascular hemolysis releases free-plasma Hb10, which contains heme and is highly toxic when freely released into the body11. To maintain homeostasis and prevent toxic heme from being released, Haptoglobin (Hpt), a glycoprotein made …show more content…

Hemopexin (Hpx), produced by hepatic cells and also by other tissues (such as skin, lung, and kidney)18, is a heme-binding plasma glycoprotein19, which has the highest binding affinity to heme19. In the event of hemolytic anemia, Hpx immediately takes over the role of Hpt and continues to protect against toxic heme18. Furthermore, enzymes break down the Hb into heme, which instantly binds with Hpx. Afterwards, CD91/LRP-1 carries out the Hb and Hpx proteins to the liver20, where it gets secreted and is broken down to maintain homeostasis in the body. However, failure of Hpx binding to heme or Hpt binding to Hb consequently results in the release of toxic heme, which can cause chronic and acute effects on the liver, spleen, and kidney, as well as severe inflammation, cell injury, and oxidative damage21. The most effective treatment for resolving the imbalanced populations of Hpt and Hpx are transfusions every couple of weeks21. Healthy blood is supposed to enter the SCD blood and restore the Hpt and Hpx proteins and preserve homeostasis control, however previous research has yet to investigate the protein levels before and after transfusions through ELISAs. In some cases, SCD patients only receive transfusions every 6 months; they’re known as control SCD populations. Limited research has been done regarding Hpt and Hpx levels in control SCD populations,

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