hemoglobin ss การใช้

• The association of two wild-type ?-globin subunits with two mutant ?-globin subunits forms hemoglobin S ( HbS ).
• Working with the extensive patient population of Charity Hospital, he was instrumental in the discovery of Hemoglobin SS and its relevance to Sickle Cell Disease.
• By this combination he created a two-dimensional method that enabled him to comparatively " fingerprint " the hemoglobin S and A fragments he obtained from the tryspin digest.
• How it is inherited : When both parents have the sickle cell trait ( the hemoglobin S gene ), one out of four of their children on average will have sickle cell anemia.
• Children with sickle-cell disease instead begin producing a defective form of hemoglobin called hemoglobin S which aggregates together and forms filaments that cause red blood cells to change their shape from round to sickle-shaped.
• Due to its relative stability compared to hemoglobin Gower 1 and hemoglobin S, it has been proposed as a subject for reactivation in the adult in cases of severe hemoglobinopathies in subjects for which the reactivation of hemoglobin F is contraindicated due to toxicity concerns.
• The fetal hemoglobin's reduction in the severity of the disease comes from its ability to inhibit the formation of hemoglobin aggregates within red blood cells which also contain hemoglobin S . Combination therapy with hydroxyurea and recombinant erythropoietin & mdash; rather than treatment with hydroxyurea alone & mdash; has been shown to further elevate hemoglobin F levels and to promote the development of HbF-containing F-cells.