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α Hemoglobin stabilizing protein: a erythroid molecular chaperone

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Document pages: 7 pages

Abstract: α Hemoglobin stabilizing protein (AHSP) is a erythroid specific protein, which is used as hemoglobin free protein α Chain molecular chaperone. There is strong evidence that AHSP is involved in hemoglobin synthesis and may play a role in neutralization between normal and normal β Excessive free accumulation in erythroid precursor cells of thalassemia α- Cytotoxic effect of globin subunit. Therefore, AHSP seems to be essential for normal erythropoiesis. Impaired AHSP upregulation may lead to premature death of erythrocytes and ineffective erythropoiesis. In some β- In thalassemia cases, the decreased expression of AHSP mRNA is associated with clinical variability. Studies have shown that, α- Hb variants may also damage AHSP- α- HB interaction leads to similar pathological conditionsα-thalassemia syndromes. The aim of this paper is to summarize current information concerning the structure and function of AHSP, focusing on its role in normal erythropoiesis and its relevance in health and disease.

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