Sickle Cell Anemia

by Kevin Ahern, PhD

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    Well, there’s yet one more thing to understand about hemoglobin that’s really critical. And this is a disease known as sickle cell anemia which most people have heard of. Sickle cell anemia is a genetic disease and it affects specifically hemoglobin. There are multiple forms of sickle cell anemia and result form different mutations, but the most common mutation occurs by changing a glutamic acid at position number six to a valine. Now, that very minor change in the structure of a protein causes this hemoglobin to have some very unusual properties. The hemoglobin will tend in these cells to have a polymeric form when the oxygen concentration is low. This polymer of hemoglobin causes the red blood cell that contains it to change its shape. That shape change happens and causes a sickle form of the blood cell as a result of the polymerization of the hemoglobin that I’ve described. Now, you can see on this picture, at the very top, sickled cells. These are cells that have encountered that low oxygen concentration. They’ve had their hemoglobin polymerized and the cell shape has changed accordingly. Well, sickled cells are real problems in the body. Because as the blood cells are traveling through the body, they have to pass through capillaries. Capillaries are where most of the oxygen exchange actually occurs. Rounded red blood cells make it through those capillaries very readily. But the capillaries are where the oxygen is being taken away. It’s in those capillaries where the oxygen concentration will be the lowest, and it’s in those capillaries where the sickling of the red blood cell will happen in a person who has this disease. When that happens, these sickle shapes form, they block the capillaries. The sickled cells get stuck inside of the red blood cells. Now,...

    About the Lecture

    The lecture Sickle Cell Anemia by Kevin Ahern, PhD is from the course Amino Acid Metabolism.

    Included Quiz Questions

    1. The complications arise from sickled red blood cells getting stuck in capillaries.
    2. Sickling of blood cells occurs upon binding of oxygen.
    3. The disease gives most protection from malaria when homozygous.
    4. All of the answers are true.
    5. None of the answers are true.

    Author of lecture Sickle Cell Anemia

     Kevin Ahern, PhD

    Kevin Ahern, PhD

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