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The inability to produce functional beta-globin results in an imbalance of alpha- and beta-globin subunits. The accumulation of free alpha-globin subunits causes red blood cells to lyse (burst) or undergo apoptosis (Figure 3). The extra alpha-globin subunits are unable to correctly fold into a hemoglobin protein complex and instead form structures called alpha-hemichromes. The alpha-hemichromes are unable to bind oxygen molecules and become insoluble in the cytoplasm of the red blood cells. The
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