basic FUNCTION
| abundant, erythroid-specific protein that forms a stable complex with free alpha-haemoglobin but not with beta-haemoglobin or haemoglobin A (alpha(2)beta(2)) |
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required for normal erythropoiesis, probably acting to block the deleterious effects of free alpha-haemoglobin precipitation |
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acts as a molecular chaperone to stabilize nascent alpha-globin for HbA assembly |
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potential modifier of beta-thalassemia by virtue of its ability to detoxify excess free alpha-globin |
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inhibits cellular production of the reactive oxygen species |
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reversibly binds nascent alpha globin to maintain its native structure and facilitate its incorporation into hemoglobin A |
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promotes alpha globin chain stability during human erythropoiesis |
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may function as an alpha globin -specific chaperone, and suggest an important role for nascent alpha chain in guiding Hb assembly by stabilizing nascent beta chain and inhibiting off-pathway self-association of beta-haem |