Definition: two abnormal Hb's, both with the substitution of a valyl residue for a glutamyl residue at the sixth position of the ß chain as in Hb S, and in addition, each has a second substitution of an asparaginyl residue for an aspartyl residue at position 73 of the ß chain; both types cause sickling of erythrocytes similar to Hb S.
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