Literature DB >> 5032531

Equal synthesis of - and -globin chains in erythroid precursors in heterozygous -thalassemia.

Y W Kan, D G Nathan, H F Lodish.   

Abstract

In patients with heterozygous beta-thalassemia, the beta/alpha synthetic ratio in marrow erythroid cells incubated in vitro is 1, whereas in reticulocytes the ratio is 0.5. These ratios reflect the equal synthesis of the two chains on the polyribosomes of the bone marrow and unequal synthesis on the polyribosomes of the peripheral blood reticulocytes. alpha- and beta-chain synthesis is also equal in marrow cells in vivo. Equal synthesis is probably due both to a decrease in alpha-chain synthesis and an increase in beta-chain synthesis in bone marrow erythroid cells and may contribute to the absence of overt hemolysis due to excess alpha-globin chain accumulation in heterozygous beta-thalassemia.

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Year:  1972        PMID: 5032531      PMCID: PMC292339          DOI: 10.1172/JCI106993

Source DB:  PubMed          Journal:  J Clin Invest        ISSN: 0021-9738            Impact factor:   14.808


  14 in total

1.  AMINO ACIDS: INCORPORATION INTO ALPHA- AND BETA-CHAINS OF HEMOGLOBIN BY NORMAL AND THALASSEMIC RETICULOCYTES.

Authors:  J D HEYWOOD; M KARON; S WEISSMAN
Journal:  Science       Date:  1964-10-23       Impact factor: 47.728

2.  The synthesis of heme and globin in the maturing human erythroid cell.

Authors:  D G NATHAN; S PIOMELLI; F H GARDNER
Journal:  J Clin Invest       Date:  1961-06       Impact factor: 14.808

3.  Excess of alpha-globin synthesis in homozygous beta-thalassemia and its removal from the red blood cell cytoplasm.

Authors:  A Bargellesi; S Pontremoli; C Menini; F Conconi
Journal:  Eur J Biochem       Date:  1968-01

Review 4.  Thalassemia: the consequences of unbalanced hemoglobin synthesis.

Authors:  D G Nathan; R B Gunn
Journal:  Am J Med       Date:  1966-11       Impact factor: 4.965

5.  Beta thalassemia and translation of globin messenger RNA.

Authors:  D G Nathan; H F Lodish; Y W Kan; D Housman
Journal:  Proc Natl Acad Sci U S A       Date:  1971-10       Impact factor: 11.205

6.  Intracellular loss of free alpha chains in beta thalassemia.

Authors:  A Bank; J V O'Donnell
Journal:  Nature       Date:  1969-04-19       Impact factor: 49.962

7.  Changing rates of globin chain synthesis during erythroid cell maturation in thalassemia.

Authors:  A S Braverman; A Bank
Journal:  J Mol Biol       Date:  1969-05-28       Impact factor: 5.469

8.  Peptide analysis of the inclusions of erythroid cells in beta-thalassemia.

Authors:  P Fessas; D Loukopoulos; A Kaltsoya
Journal:  Biochim Biophys Acta       Date:  1966-08-24

9.  Globin synthesis in thalassaemia: an in vitro study.

Authors:  D J Weatherall; J B Clegg; M A Naughton
Journal:  Nature       Date:  1965-12-11       Impact factor: 49.962

10.  Heterozygous Beta thalassemia: balanced globin synthesis in bone marrow cells.

Authors:  E Schwartz
Journal:  Science       Date:  1970-03-13       Impact factor: 47.728

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  12 in total

1.  Globin synthesis in fractionated Normoblasts of beta-thalassemia heterozygotes.

Authors:  W G Wood; G Stamatoyannopoulos
Journal:  J Clin Invest       Date:  1975-03       Impact factor: 14.808

Review 2.  Protein quality control during erythropoiesis and hemoglobin synthesis.

Authors:  Eugene Khandros; Mitchell J Weiss
Journal:  Hematol Oncol Clin North Am       Date:  2010-12       Impact factor: 3.722

3.  Imbalanced globin chain synthesis in heterozygous beta-thalassemic bone marrow.

Authors:  G Chalevelakis; J B Clegg; D J Weatherall
Journal:  Proc Natl Acad Sci U S A       Date:  1975-10       Impact factor: 11.205

4.  Integrated protein quality-control pathways regulate free α-globin in murine β-thalassemia.

Authors:  Eugene Khandros; Christopher S Thom; Janine D'Souza; Mitchell J Weiss
Journal:  Blood       Date:  2012-03-16       Impact factor: 22.113

5.  Letter: Anaemia in beta-thalassaemic carriers.

Authors:  G Castaldi; G Zavagli; G Ambroso; B Dallapiccola; F Trotta
Journal:  Br Med J       Date:  1974-03-16

6.  Globin chain synthesis in sickle beta-thalassaemic bone marrow and reticulocytes.

Authors:  S Ladas; G Chalevelakis; C Lyberatos; E Vaidakis; G Arapakis
Journal:  J Med Genet       Date:  1979-08       Impact factor: 6.318

7.  An RNAi therapeutic targeting Tmprss6 decreases iron overload in Hfe(-/-) mice and ameliorates anemia and iron overload in murine β-thalassemia intermedia.

Authors:  Paul J Schmidt; Iva Toudjarska; Anoop K Sendamarai; Tim Racie; Stuart Milstein; Brian R Bettencourt; Julia Hettinger; David Bumcrot; Mark D Fleming
Journal:  Blood       Date:  2012-12-06       Impact factor: 22.113

8.  Hemoglobin messenger RNA from human bone marrow. Isolation and translation in homozygous and heterozygous beta-thalassemia.

Authors:  A W Nienhuis; P H Canfield; W F Anderson
Journal:  J Clin Invest       Date:  1973-07       Impact factor: 14.808

9.  Free alpha-globin pool in human bone marrow.

Authors:  F M Gill; E Schwartz
Journal:  J Clin Invest       Date:  1973-12       Impact factor: 14.808

10.  Translational control of hemoglobin synthesis in thalassemic bone marrow.

Authors:  G Cividalli; D G Natham; H F Lodish
Journal:  J Clin Invest       Date:  1974-04       Impact factor: 14.808

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