Band 3 protein disorder in one family with G6PD enzyme deficiency

Abstract

Purpose: Proteins found in complex membrane structure determines its stability and flexibility. The structural defect and deficiencies observed on erythrocyte membrane cause hemolytic anemias. In this study the erythrocyte membrane proteins of a family having G6PD enzyme deficiency were studied to investigate its possible contribution to hemolysis. Methods: The enzyme activity was measured by using Beutler method and red cell membrane proteins were investigated by SDS-PAGE in 8.3% gels according to Fairbanks et al. Results: G6PD enzyme deficiency was found in all members of family, except for the father. While as an erythrocyte membrane protein band 3 protein deficiency was observed in the mother, father and two children, but one child was found to be normal. Also the mother and 5 years old son were sickle cell carrier, and the father has ?-thalassemia-2 (?3.7) Conclusion: The results showed that there may be associations between erythrocyte membrane proteins and hemolytic anemias such as G6PD deficiency and hemoglobinopathies

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