Frequently asked question: What is wrong with the hemoglobin in cases of sickle cell disease?

When you have sickle cell disease, your hemoglobin is atypical (hemoglobinopathy). One of the hemoglobin chains, i.e. the beta globin chain, is structured differently. This is caused by a change (mutation) in the beta globin gene. As a result of this mutation, the hemoglobin has a different shape. This abnormal type of hemoglobin is called hemoglobin S (where the S stands for Sickle cell). The problem is that hemoglobin S (HbS) potentially binds to other HbS in the red blood cell, causing the red blood cell to become abnormally shaped, namely the shape of a sickle. Sickle-shaped red blood cells are stickier and less flexible than normal red blood cells.

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