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PMID: 1054838 Published · ppublish English Journal Article Research Support, U.S. Gov't, P.H.S.

Further evidence of a quantitative deficiency of chain-specific globin mRNA in the thalassemia syndromes.

Proceedings of the National Academy of Sciences of the United States of America ·Vol. 72 ·No. 2 ·1975-02-00 ·Pages 567-71

Kazazian HH, Ginder GD, Snyder PG, Van Beneden RJ, Woodhead AP

Abstract

Formamide gel electrophoresis separates the mRNA fraction from reticulocyte polyribosomes of adult humans into two major RNA species with migratory rates identical to those of the alpha- and beta-globin mRNAs of the rabbit. That these two RNAs of human origin are the globin mRNAs is further supported by the deficiency of the presumed beta mRNA in reticulocyte polyribosomes of fetuses and premature infants, whose cells make gamma chains in preference to beta chains. The globin mRNAs of reticulocyte polyribosomes from patients with hematological disorders were estimated by scanning the stained formamide gels. In contrast to individuals with either hemolytic anemia without hemoglobinopathy or sickle cell anemia who had beta mRNA to alpha mRNA ratios of approximately one, a patient with Hb S-beta-thalassemia had a ratio of beta mRNA to alpha mRNA of 0.75 while two subjects with homozygous beta-thalassemia had severe deficiencies of beta mRNA. Conversely, a patient with alpha-thalassemia (Hb H disease) had a ratio of beta mRNA to alpha mRNA on reticulocyte polyribosomes of 6. These data provide further evidence of a quantitative deficiency of chain-specific globin mRNA in patients with the thalassemia syndromes.

MeSH Terms
Adult Child Electrophoresis, Polyacrylamide Gel Female Globins/biosynthesis Heterozygote Homozygote Humans Male Polyribosomes/metabolism RNA, Messenger/blood,deficiency Reticulocytes/metabolism,ultrastructure Thalassemia/blood,genetics
Chemicals
RNA, Messenger Globins
Authors & Affiliations
5 authors, click to expand affiliations / ORCID
Kazazian H H
Ginder G D
Snyder P G
Van Beneden R J
Woodhead A P
References (38)
38 references, click to expand
  1. HUMAN HEMOGLOBIN GENE LINKAGE: REPORT OF A FAMILY WITH HEMOGLOBIN B2, HEMOGLOBIN S, AND BETA THALASSEMIA, INCLUDING A PROBABLE CROSSOVER BETWEEN THALASSEMIA AND DELTA LOCI.
    Am J Hum Genet. 1965 Mar;17:125-32 PMID: 14262130
  2. Thalassemia minor associated with hemoglobin-B2 heterozygosity. A family report.
    Blood. 1961 Jun;17:747-57 PMID: 13716727
  3. Purification of biologically active globin messenger RNA by chromatography on oligothymidylic acid-cellulose.
    Proc Natl Acad Sci U S A. 1972 Jun;69(6):1408-12 PMID: 4504350
  4. Separation of alpha- and beta-globin messenger RNAs.
    Nat New Biol. 1972 Aug 9;238(84):166-9 PMID: 4505983
  5. In vitro synthesis of DNA components of human genes for globins.
    Nat New Biol. 1972 Feb 9;235(58):167-9 PMID: 4334194
  6. In vitro synthesis of DNA complementary to rabbit reticulocyte 10S RNA.
    Nat New Biol. 1972 Feb 9;235(58):163-7 PMID: 4334193
  7. Induction of -globin synthesis in the -thalassaemia of Ferrara.
    Nat New Biol. 1972 Jul 19;238(81):83-7 PMID: 4505416
  8. Decreased globin messenger RNA in thalassemia detected by molecular hybridization.
    Proc Natl Acad Sci U S A. 1973 Jun;70(6):1886-90 PMID: 4124307
  9. Quantitative deficiency of chain-specific globin messenger ribonucleic acids in the thalassemia syndromes.
    Proc Natl Acad Sci U S A. 1973 Jun;70(6):1809-13 PMID: 4124305
  10. Hemoglobin messenger RNA from human bone marrow. Isolation and translation in homozygous and heterozygous beta-thalassemia.
    J Clin Invest. 1973 Jul;52(7):1735-45 PMID: 4718962
  11. The molecular weight of rabbit globin messenger RNA's.
    FEBS Lett. 1973 Mar 15;30(3):301-4 PMID: 4700749
  12. Decreased globin messenger RNA activity associated with polyribosomes in thalassaemia.
    Nat New Biol. 1973 Feb 14;241(111):209-11 PMID: 4512586
  13. Absence of messenger RNA for beta globin chain in beta(0) thalassaemia.
    Nature. 1974 Feb 8;247(5440):379-81 PMID: 4817859
  14. Decreased beta globin mRNA activity in bone marrow cells in homozygous and heterozygous beta thalassaemia.
    Nat New Biol. 1973 Aug 29;244(139):280-1 PMID: 4517009
  15. In vitro synthesis of DNA complementary to purified rabbit globin mRNA (RNA-dependent DNA polymerase-reticulocyte-hemoglobin-density gradient centrifugation-oligo(dT) primer).
    Proc Natl Acad Sci U S A. 1972 Jan;69(1):264-8 PMID: 4333043
  16. Isolation and translation of hemoglobin messenger RNA from thalassemia, sickle cell anemia, and normal human reticulocytes.
    J Clin Invest. 1971 Nov;50(11):2458-60 PMID: 5096528
  17. Separation of alpha- and beta-globin messenger RNAs by formamide gel electrophoresis.
    Biochem Biophys Res Commun. 1974 Aug 5;59(3):1053-61 PMID: 4415727
  18. The separation and identification of alpha- and beta-globin messenger ribonucleic acids.
    J Biol Chem. 1974 Aug 25;249(16):5290-5 PMID: 4855083
  19. Globin synthesis in thalassaemia: an in vitro study.
    Nature. 1965 Dec 11;208(5015):1061-5 PMID: 5870556
  20. Molecular weight determination of nucleic acids by gel electrophoresis in non-aqueous solution.
    Nat New Biol. 1972 Jan 26;235(56):108-10 PMID: 4551278
  21. The severe form of alpha thalassaemia is caused by a haemoglobin gene deletion.
    Nature. 1974 Oct 4;251(5474):389-92 PMID: 4138824
  22. Protein synthesis directed by encephalomyocarditis virus RNA: properties of a transfer RNA-dependent system.
    Proc Natl Acad Sci U S A. 1971 Sep;68(9):2303-7 PMID: 4332253
  23. Regulation of hemoglobin beta-chain synthesis in bone marrow erythroid cells by alpha chains.
    Proc Natl Acad Sci U S A. 1973 Dec;70(12):3405-9 PMID: 4519634
  24. Thalassemia in black americans.
    Ann N Y Acad Sci. 1974;232(0):125-34 PMID: 4528763
  25. Haemoglobin Icaria, a new chain-termination mutant with causes alpha thalassaemia.
    Nature. 1974 Sep 20;251(5472):245-7 PMID: 4422784
  26. Genetic lesion in homozygous alpha thalassaemia (hydrops fetalis).
    Nature. 1974 Oct 4;251(5474):392-3 PMID: 4424635
  27. Hemoglobin A synthesis in the developing fetus.
    N Engl J Med. 1973 Jul 12;289(2):58-62 PMID: 4710406
  28. Electrophoretic characterization of bacterial polyribosomes in agarose-acrylamide composite gels.
    J Mol Biol. 1969 Apr 14;41(1):139-47 PMID: 4979520
  29. Hb-S, beta thalassemia and Hb-A-2 (B-2) in a family with evidence of a crossover between beta and delta loci.
    Acta Genet Stat Med. 1965;15(3):371-7 PMID: 4957363
  30. Haemoglobin Constant Spring--a chain termination mutant?
    Nature. 1971 Dec 10;234(5328):337-40 PMID: 4944483
  31. Adult hemoglobin synthesis by reticulocytes from the human fetus at midtrimester.
    Science. 1971 Nov 12;174(4010):698-702 PMID: 5123417
  32. Defect in messenger RNA for human hemoglobin synthesis in beta thalassemia.
    J Clin Invest. 1971 Dec;50(12):2755-60 PMID: 5129324
  33. Mammalian cell-free protein synthesis directed by viral ribonucleic acid.
    Eur J Biochem. 1970 Dec;17(2):328-38 PMID: 5500401
  34. Hemoglobin messenger ribonucleic acid. Synthesis of 9S and ribosomal ribonucleic acid during erythroid cell development.
    Biochemistry. 1969 Jul;8(7):3000-5 PMID: 5808350
  35. Isolation of an RNA with the properties of haemoglobin messenger.
    Nature. 1969 Mar 29;221(5187):1217-22 PMID: 5773836
  36. Haemoglobin synthesis in alpha-thalassaemia (haemoglobin H disease).
    Nature. 1967 Sep 16;215(5107):1241-3 PMID: 6052721
  37. Abnormal human haemoglobins. Separation and characterization of the alpha and beta chains by chromatography, and the determination of two new variants, hb Chesapeak and hb J (Bangkok).
    J Mol Biol. 1966 Aug;19(1):91-108 PMID: 5967288
  38. Asymmetrical incorporation of amino acids in the alpha and beta chains of hemoglobin synthesized by thalassemic reticulocytes.
    J Lab Clin Med. 1965 Sep;66(3):476-82 PMID: 5835972
Article Info
Journal
Proceedings of the National Academy of Sciences of the United States of America
Abbr.
Proc Natl Acad Sci U S A
ISSN
0027-8424
DOI
10.1073/pnas.72.2.567
Published
1975-02-00
Pages
567-71
Language
English
Region
United States
NLM ID
7505876
PMCID
PMC432354
Subset
IM
External Links
PubMed source DOI: 10.1073/pnas.72.2.567 PMC Full Text View journal details
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