BACKGROUND: Hemoglobin (Hb) Groene Hart [␣119 (H2)Pro3 Ser (␣1)], also known as Hb Bernalda, is a nondeletional ␣-thalassemic Hb variant that is frequent in southern Italy and North Africa. This variant is not supposed to be produced in the erythrocytes of carriers. The ␣-thalassemic behavior of this variant has been explained as an impaired interaction between the ␣-globin chain and the ␣-Hb-stabilizing protein.
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