Alpha hemoglobin stabilizing protein: Its causal relationship with the severity of beta thalassemia.

摘要: Thalassemia major is characterized by anemia, iron overload and cellular damage. The severity of symptoms correlates with the alpha/non-alpha globin imbalance proportional to magnitude alpha chain excess. Alpha hemoglobin stabilizing protein (AHSP), erythroid specific chaperone, stabilizes free chains, prevents formation reactive oxygen radicals. Though AHSP expression has been linked beta thalassemia, its role as a probable genetic modifier disease severity, still not unequivocally established. In present study, level chaperone seen vary in regularly transfused thalassemia patients, being underexpressed 64% cases, upregulated 16% comparable controls 20% cases. This discrepancy may be attributed degree DNA damage, % HbF, number nucleated RBCs peripheral blood these patients. Results reveal that decrease pool, hence repertoire unbound iron, due elevated HbF and/or presence results upregulation gene.