Altered sialic acid contents of red blood cell membrane preparations in homozygous beta-thalassemia.
In the present work the sialic acid content of the red blood cell ghost membranes was measured in blood specimens obtained from 18 patients with beta-thalassemia major and 10 controls. The sialic acid-protein weight ratio was reduced in comparison with normal mean value only in four patients who had undergone splenectomy several years ago. The remaining 14 unsplenectomized patients showed a sialic acid/protein ratio that was 50% higher than normal (P less than 0.001). The titratable sulphydryl groups were also measured in red blood cell membrane preparations. SH groups content was reduced in patients with beta-thalassemia major, and a dramatic decrease (-60%) was observed in patients after splenectomy.
D000293:Adolescent; D000328:Adult; D001798:Blood Proteins / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D004910:Erythrocyte Membrane / Q000378:metabolism*; D004912:Erythrocytes / Q000378:metabolism*; D005260:Female; D006720:Homozygote; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012794:Sialic Acids / Q000097:blood*; D013156:Splenectomy; D013438:Sulfhydryl Compounds / Q000097:blood; D013789:Thalassemia / Q000097:blood* / Q000235:genetics
D001798:Blood Proteins; D012794:Sialic Acids; D013438:Sulfhydryl Compounds