Absence of functional beta-globin messenger RNA in Kurdish Jews with beta0-thalassemia.
Human globin messenger RNA was isolated from reticulocytes of four Jewish patients of Kurdish origin with homozygous beta0-thalassemia. On translation in the wheat-germ cell-free system, messenger RNA from these patients directed extensive synthesis of alpha- and gamma-globin chains, but synthesis of beta-globin chains was not detectable. In contrast, nonthalassemic human globin messenger RNA directed the synthesis of essentially equimolar amounts of alpha- and beta-globin. The patterns of globin synthesized by beta0-thalassemic messenger RNA in the cell-free system were virtually identical to the patterns of globin synthesized in peripheral blood cells of these patients. beta0-thalassemic messenger RNA similarly failed to direct any detectable beta-globin synthesis in a micrococcal nuclease-treated rabbit reticulocyte lysate, even in the presence of an excess of purified eukaryotic initiation factor 2. These results strongly suggest that functional messenger RNA for beta-globin chains is absent in Kurdish Jews with homozygous beta0-thalassemia.
D002474:Cell-Free System; D005006:Ethnicity; D005914:Globins / Q000096:biosynthesis*; D006720:Homozygote; D006801:Humans; D007557:Israel; D007585:Jews; D007930:Leucine / Q000378:metabolism; D008836:Micrococcal Nuclease / Q000494:pharmacology; D010448:Peptide Initiation Factors / Q000494:pharmacology; D014176:Protein Biosynthesis / Q000187:drug effects; D011506:Proteins / Q000494:pharmacology; D012333:RNA, Messenger / Q000097:blood* / Q000494:pharmacology; D012156:Reticulocytes / Q000378:metabolism; D013789:Thalassemia / Q000097:blood*; D014908:Triticum
D010448:Peptide Initiation Factors; D011506:Proteins; D012333:RNA, Messenger; D005914:Globins; D008836:Micrococcal Nuclease; D007930:Leucine