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The hemoglobin holoenzyme (active enzyme) is a complex of an iron ion and 4 subunits- 2 hemoglobin alpha and two hemoglobin beta. People with sickle cell disease have mutated beta hemoglobins. The mutation is a single nucleotide exchange, that changes the 6th amino acid of  protein primary struture - a Glutamic acid into Valine. This one alteration changes the tree dimentional structure of hemoglobin beta so, that it forms lond fibres that disrupt the normal circular form of the red blood cells.
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