Comparison of erythrocyte antioxidative enzyme activities between two types of haemoglobin H disease.
The activities of erythrocyte antioxidative enzymes were measured in two groups of patients with different genotypes of haemoglobin (Hb) H disease: 21 with alpha-thalassaemia 1 or alpha-thalassaemia 2 (alpha-thalassaemia 1/2) and 21 with alpha-thalassaemia 1/Hb Constant Spring (HbCS). They were compared with 21 normal subjects. Both genotypes of Hb H disease had increased activities of erythrocyte superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), and catalase when compared with those of controls. Comparison of the two genotypes showed that subjects with alpha-thalassaemia 1/Hb CS, the more severe disease, had higher SOD and GSH-Px activities but lower catalase activity than those with alpha-thalassaemia 1/2. This indicates that there are compensatory mechanisms in Hb H erythrocytes to cope with increased generation of oxygen free radicals as a result of increased excess beta chain.
D002374:Catalase / Q000097:blood*; D004912:Erythrocytes / Q000201:enzymology*; D005838:Genotype; D005979:Glutathione Peroxidase / Q000097:blood*; D006455:Hemoglobins, Abnormal; D006801:Humans; D013482:Superoxide Dismutase / Q000097:blood*; D013789:Thalassemia / Q000097:blood / Q000201:enzymology*
D006455:Hemoglobins, Abnormal; C035541:Hemoglobin Constant Spring; D002374:Catalase; D005979:Glutathione Peroxidase; D013482:Superoxide Dismutase