Sickle Cell Anemia. Sickle Cell anemia is an inherited red blood cell disorder. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen.
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Sickle Cell anemia is an inherited red blood cell disorder. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen.
Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This can cause pain, damage and a low blood count, or anemia.
The change in cell structure arises from a change in
the structure of hemoglobin.
A single change in an amino acid causes hemoglobin
Normal hemoglobin has
four subunits that each
contain an oxygen binding
Biswal, B. K., Vijayan, M.:
Structures of Human Oxy-
and Deoxyhaemoglobin at
Different Levels of Humidity:
Variability in the T State
Acta Crystallogr., Sect.D
58 pp. 1155 (2002)
A single mutation in
hemoglobin results in a
binding of one protein
Padlan, E. A., Love, W. E.: Refined crystal structure of deoxyhemoglobin S. I. Restrained least-squares refinement at 3.0-Å resolution. J Biol Chem 260 pp. 8272 (1985)
deoxy Hb (CO2)
deoxy Hb (CO2)
Strong binding state
Weak binding state
Hemoglobin is composed of two a and two b
subunits whose structures resemble myoglobin.
Eaton et al. Nature Struct. Biol. 1999, 6, 351
Normal hemoglobin (Hb A)
Sickle cell hemoglobin (Hb S)