Sickle Cell Anemia is a genetic disease that causes many health problems for its hosts. Sickle Cell disease manifests when you inherit two “S” genes from your parents. Having Sickle Cell Anemia, subjects you to a number of symptoms the most prominent of which are severe episodes of pain known as vaso-occlusive crisis. These episodes occur because the transport of oxygen in our bodies is halted. Our Erythrocytes clump together and adhere to our endothelium blocking the flow of blood in our veins which prevents oxygen from being relayed to different tissues and organs. This Crisis is one of the major if not the major complication of this disease and thus a lot of research has been conducted to help better understand and combat it.
My laboratory focuses on these vaso-occlusive episodes and has been trying to understand its mode of operation in hopes of using that knowledge to counteract these episodes. In other words, the Telen lab wants to understand what changes occur within these erythrocytes that causes them to, all of a sudden, adhere to one another and to our endothelium.
My project in particular is being carried out in conjunction with my mentor Martha Delahunty. Studies have shown that ATP can act as a vaso-dilator when released from our red blood cells. Which is ideal to prevent adhesion from halting blood flow. However, Sickle Cell patients have Erythrocytes with hemoglobin “S” as opposed to hemoglobin A and this somehow causes these red blood cells to both produce and export ATP less efficiently. Over the past three weeks, I’ve been gathering lots of Sickle Cell blood samples and extracting the ATP from them. I divide each sample of blood I get and place them in three different buffers. While one of these buffers acts as a control, the other two contain ingredients believed to better promote ATP formation and encourage ATP export. I then use what’s known as an ATP bioluminescence assay to determine how much ATP each of these samples in their respective buffers carry. Once this is completed I’ll run the same tests but instead will determine the levels of extracellular ATP. If there’s sufficient time once I’ve finished assaying these sickle cell samples and compared them to regular blood samples, I’ll subject more sickle and regular blood samples to hypoxic (oxygen deficient) conditions because it’s also believed that when cells are in a hypoxic state they more urgently release ATP to re-oxygenate. The implications of this work lie in the potential for treatment of sickle cell patients. If these buffers that we place our blood samples in increase ATP production and export, then the same ingredients that make up the buffers can be incorporated into some form of drug therapy.
