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" ... In making hemoglobin, which carries oxygen in our red blood cells, a defect can occur when the amino acid glutamate is replaced by valine. This simple substitution can cause the red blood cell to sometimes collapse, assuming the characteristic sickle shape. The red blood cells, normally very elastic and able to conform to the shape of tiny capillary blood vessels, become rigid and can block the blood vessels, depriving tissue of oxygen and resulting in severe pain. ... "