Basics of Sickle Cell Who suffers from it? What is Hemoglobin? Fiber formation Protein

Sickle cell disease is an inherited blood disorder that is caused by the presence of a mutation in the gene that encodes for the protein, hemoglobin.  Hemoglobin is the protein, which carries oxygen to the tissues.  For patients suffering from sickle cell disease, this genetic mutation causes the hemoglobin protein to be altered; specifically it becomes sticky on the surface of the protein.  Different sickle cell hemoglobin molecules stick together and form long fibers or rods.  These fibers distort the shape of the red blood cells into sickles and cause these cells to be less flexible or stiffer in traveling through the red blood vessels.  The sickled shape impedes the travel of the red blood cells and as a result, organs and tissues become deprived of oxygen, which can lead to periodic pain, damage to organs and stroke.  (Red blood cells) RBC's, which have undergone the sickling process, are less malleable and rupture more quickly than regular RBC's leading to anemia.

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