Translational control of hemoglobin synthesis in thalassemic bone marrow. 1974

G Cividalli, and D G Natham, and H F Lodish

Previous studies of beta-thalassemic reticulocytes have implied a decreased amount of functional beta-mRNA but unimpaired translation of the beta-mRNA present. However, the beta/alpha synthetic ratios in beta-thalassemic marrow are higher than those observed in reticulocytes of the same patients. This could imply that marrow cells contain an abnormally functioning beta-mRNA no longer active in reticulocytes. To test the function of mRNA found in marrow, intact cells were incubated with [(35)S]methionine and the relative amounts of nascent alpha- and beta-chains on polysomes of different sizes were measured by tryptic digestion and determination of the specific activities of the respective peptides. Results showed that in normal and beta-thalassemic marrow, as well as in reticulocytes, beta-chain production, though deficient, occurs predominantly on larger polysomes than the production of alpha-chains. In one patient with severe thalassemia and very little production of beta-chains in marrow or reticulocytes, delta-chain synthesis was found predominantly on larger polysomes than alpha-chain synthesis. These results indicate that in beta-thalassemic as well as in nonthalassemic marrow and reticulocytes, each beta- and delta-mRNA initiates protein synthesis at a rate faster than does each alpha-mRNA, and suggest that the beta-mRNA in contact with polyribosomes is normally functioning but quantitatively deficient in beta-thalassemic marrow as well as in reticulocytes. No translational defect was detected in a similar study performed in reticulocytes of a patient with hemoglobin H disease, suggesting a normally functioning mRNA in contact with polyribosomes in this condition as well. In both thalassemias, unbalanced synthesis of alpha- and beta-chains was more pronounced on polysomes than in completed chains. This difference possibly reflects a compensatory delay in translation of the nonthalassemic chain, which is present in excess.

UI MeSH Term Description Entries
D008715 Methionine A sulfur-containing essential L-amino acid that is important in many body functions. L-Methionine,Liquimeth,Methionine, L-Isomer,Pedameth,L-Isomer Methionine,Methionine, L Isomer
D011132 Polyribosomes A multiribosomal structure representing a linear array of RIBOSOMES held together by messenger RNA; (RNA, MESSENGER); They represent the active complexes in cellular protein synthesis and are able to incorporate amino acids into polypeptides both in vivo and in vitro. (From Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed) Polysomes,Polyribosome,Polysome
D012156 Reticulocytes Immature ERYTHROCYTES. In humans, these are ERYTHROID CELLS that have just undergone extrusion of their CELL NUCLEUS. They still contain some organelles that gradually decrease in number as the cells mature. RIBOSOMES are last to disappear. Certain staining techniques cause components of the ribosomes to precipitate into characteristic "reticulum" (not the same as the ENDOPLASMIC RETICULUM), hence the name reticulocytes. Reticulocyte
D001853 Bone Marrow The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. Marrow,Red Marrow,Yellow Marrow,Marrow, Bone,Marrow, Red,Marrow, Yellow
D002499 Centrifugation, Density Gradient Separation of particles according to density by employing a gradient of varying densities. At equilibrium each particle settles in the gradient at a point equal to its density. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed) Centrifugations, Density Gradient,Density Gradient Centrifugation,Density Gradient Centrifugations,Gradient Centrifugation, Density,Gradient Centrifugations, Density
D004586 Electrophoresis An electrochemical process in which macromolecules or colloidal particles with a net electric charge migrate in a solution under the influence of an electric current. Electrophoreses
D005914 Globins A superfamily of proteins containing the globin fold which is composed of 6-8 alpha helices arranged in a characterstic HEME enclosing structure. Globin
D006447 Hemoglobin H An abnormal hemoglobin composed of four beta chains. It is caused by the reduced synthesis of the alpha chain. This abnormality results in ALPHA-THALASSEMIA.
D006453 Hemoglobinopathies A group of inherited disorders characterized by structural alterations within the hemoglobin molecule. Hemoglobinopathy
D006454 Hemoglobins The oxygen-carrying proteins of ERYTHROCYTES. They are found in all vertebrates and some invertebrates. The number of globin subunits in the hemoglobin quaternary structure differs between species. Structures range from monomeric to a variety of multimeric arrangements. Eryhem,Ferrous Hemoglobin,Hemoglobin,Hemoglobin, Ferrous

Related Publications

G Cividalli, and D G Natham, and H F Lodish
January 1974, Annals of the New York Academy of Sciences,
G Cividalli, and D G Natham, and H F Lodish
January 1969, Cold Spring Harbor symposia on quantitative biology,
G Cividalli, and D G Natham, and H F Lodish
October 1975, Proceedings of the National Academy of Sciences of the United States of America,
G Cividalli, and D G Natham, and H F Lodish
August 1969, Biochemical and biophysical research communications,
G Cividalli, and D G Natham, and H F Lodish
November 1974, Annals of the New York Academy of Sciences,
G Cividalli, and D G Natham, and H F Lodish
March 1970, Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.),
G Cividalli, and D G Natham, and H F Lodish
February 1999, Blood,
G Cividalli, and D G Natham, and H F Lodish
January 1969, Cold Spring Harbor symposia on quantitative biology,
G Cividalli, and D G Natham, and H F Lodish
December 1979, The Journal of biological chemistry,
G Cividalli, and D G Natham, and H F Lodish
August 1992, Bone marrow transplantation,
Copied contents to your clipboard!