Date of Award

Spring 4-28-2017

Document Type

Thesis

Primary Advisor

Dr. Aimee Franklin

Abstract

Sickle cell anemia is a disease that affects red blood cells, specifically the hemoglobin protein. An amino acid mutation in the gene that encodes β-globin leads to malformation of the β subunit of hemoglobin.3, Valine becomes glutamic acid in the mutated β-globin gene.9 The mutation malformation leads to the red blood cells becoming sickle shaped, or crescent shaped.3,4 The sickle shape of red blood cells in individuals with sickle cell disease leads to vaso-occlusive crisis.9 Vaso-occlusive crisis includes complications such as blood clotting, chronic pain, organ failure, organ death, and possibly early mortality.9 The Centers for Disease Control and Prevention reported that there are approximately 100,000 Americans who have sickle cell disease as of their August 2016 records.21 One of every 365 African-Americans, and one in every 16,300 Hispanic-Americans have sickle cell anemia.21 There are therapies and treatments that have already been developed based on the knowledge gained regarding the disease and its complications. Currently, hydroxyurea therapy is the most widely accepted and used treatment for individuals with sickle cell disease with proven relief of vaso-occlusive crisis complications. However, with hydroxyurea therapy there are several unwanted effects that have an impact on the patients, such as jaundice, blood clotting, and usual bleeding just to name a few.44 Studies have noted an upregulation of the glycolytic enzyme, fructose-bisphosphate aldolase (aldolase) in patients who have undergone hydroxyurea therapy. Aldolase is an important enzyme that plays a role in energy conversion. With this in mind, I propose that sickle cell disease can be treated with an increase in aldolase by myoblast treatment and/or higher fructose consumptions thereby reducing the complications that come with the disease, as well as, drastically eliminating the unwanted effects that come with hydroxyurea therapy.

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