scholarly journals Globin Messenger RNA in Hemoglobin H Disease

Blood ◽  
1973 ◽  
Vol 42 (6) ◽  
pp. 825-833 ◽  
Author(s):  
Edward J. Benz ◽  
Paul S. Swerdlow ◽  
Bernard G. Forget
Keyword(s):  
Messenger Rna ◽  
Free System ◽  
Cell Free System ◽  
Globin Chains ◽  
Great Excess ◽  
Mouse Ascites ◽  
Α Chain ◽  
Globin Synthesis ◽  
Chain Synthesis ◽  
Hemoglobin H Disease

Abstract Functional messenger RNA (mRNA) for human globin synthesis was isolated from reticulocytes of each of two patients with hemoglobin H disease. The RNA was tested for its capacity to direct globin synthesis in a messenger RNA-dependent cell-free system derived from Krebs Type II mouse ascites tumor cells. In each case, hemoglobin H disease mRNA directed the synthesis of a great excess of β-globin chains relative to α-globin chains of hemoglobin A. The β/α synthetic ratios obtained in the cell-free system at saturating concentrations of mRNA were >22 and >15, respectively, for the two hemoglobin H disease mRNA preparations, whereas the β/α synthetic ratios obtained by incubation of intact reticulocytes from these same patients were 2.6 and 2.8, respectively. The β/α synthetic ratio obtained in the cell-free system did not vary when lower concentrations of hemoglobin H disease mRNA were used. A marked decrease in the amount of functional α-globin-chain mRNA relative to β-chain mRNA is therefore associated with the decreased α-chain synthesis observed in hemoglobin H disease. This decrease in α-chain-specific mRNA activity is greater than expected from the β/α synthetic ratio of intact reticulocytes in hemoglobin H disease.

scholarly journals Absence of functional messenger RNA activity for beta globin chain synthesis in beta 0-thalassemia

Blood ◽  
1975 ◽  
Vol 45 (1) ◽  
pp. 1-10
Author(s):  
EJ Jr Benz ◽  
PS Swerdlow ◽  
BG Forget
Keyword(s):  
Messenger Rna ◽  
Globin Chain ◽  
Globin Mrna ◽  
Free System ◽  
Gamma Globin ◽  
Globin Chains ◽  
Mouse Ascites ◽  
Chain Synthesis ◽  
A Chain ◽  
Globin Chain Synthesis

Functional human globin messenger RNA was isolated from reticulocytes of two patients with homozygous beta 0-thalassemia, three patients with sickle cell beta 0-thalassemia, andone patient doubly heterozygous for beta 0-thalassemia and hemoglobin Lepore. When incubated in the Krebs type II mouse ascites tumor-cell-free system, messenger RNA from these patients actively directed the synthesis of human beta s and/or alpha- and gamma-globin chains but failed to stimulate the synthesis of any beta A-chains, even though nonthalassemic human globin mRNA preparations consistently stimulated two to four times as much beta A- or beta S-globin chain synthesis as alpha-chain synthesis when incubated in the same system under the same conditions. These results strongly suggest that functional beta A-chain-specific globin mRNA is absent in beta 0-thalassemia.

scholarly journals Absence of functional messenger RNA activity for beta globin chain synthesis in beta 0-thalassemia

Blood ◽  
1975 ◽  
Vol 45 (1) ◽  
pp. 1-10 ◽  
Author(s):  
EJ Jr Benz ◽  
PS Swerdlow ◽  
BG Forget
Keyword(s):  
Messenger Rna ◽  
Globin Chain ◽  
Globin Mrna ◽  
Free System ◽  
Gamma Globin ◽  
Globin Chains ◽  
Mouse Ascites ◽  
Chain Synthesis ◽  
A Chain ◽  
Globin Chain Synthesis

Abstract Functional human globin messenger RNA was isolated from reticulocytes of two patients with homozygous beta 0-thalassemia, three patients with sickle cell beta 0-thalassemia, andone patient doubly heterozygous for beta 0-thalassemia and hemoglobin Lepore. When incubated in the Krebs type II mouse ascites tumor-cell-free system, messenger RNA from these patients actively directed the synthesis of human beta s and/or alpha- and gamma-globin chains but failed to stimulate the synthesis of any beta A-chains, even though nonthalassemic human globin mRNA preparations consistently stimulated two to four times as much beta A- or beta S-globin chain synthesis as alpha-chain synthesis when incubated in the same system under the same conditions. These results strongly suggest that functional beta A-chain-specific globin mRNA is absent in beta 0-thalassemia.

scholarly journals Asynchronous globin chain synthesis in rabbit reticulocyte lystates induced by hemin-controlled repressor

Blood ◽  
1978 ◽  
Vol 51 (4) ◽  
pp. 653-658 ◽  
Author(s):  
RS Franco ◽  
JW Hogg ◽  
OJ Martelo
Keyword(s):  
Globin Chain ◽  
Free System ◽  
Globin Chains ◽  
Reticulocyte Lysate ◽  
Chain Imbalance ◽  
Globin Synthesis ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

Abstract To define further the role of hemin-controlled repressor (HCR) in globin synthesis, we studied its effect on the synthesis of individual globin chains in a rabbit reticulocyte lysate cell-free system. In the presence of HCR there was a marked globin chain imbalance, resulting in a lowered alpha/beta ratio. These findings in vitro may have relevance to certain clinical heme deficiency states in which a similar globin chain imbalance has been observed.

scholarly journals Globin Synthesis in Iron-deficiency Anemia

Blood ◽  
1974 ◽  
Vol 44 (4) ◽  
pp. 551-555 ◽  
Author(s):  
Isaac Ben-Bassat ◽  
Miriam Mozel ◽  
Bracha Ramot
Keyword(s):  
Iron Deficiency ◽  
Iron Deficiency Anemia ◽  
Deficiency Anemia ◽  
Globin Chains ◽  
Iron Deficient ◽  
Membrane Bound ◽  
Α Chain ◽  
Globin Synthesis ◽  
Chain Synthesis ◽  
Β Chain

Abstract The α to β globin chain ratio has been determined in the peripheral red cells of 11 patients with iron deficiency anemia. The mean ratio was found to be 0.74 ± 0.07, which is significantly lower than the ratio of 0.97 ± 0.07 obtained in normals. When the stroma-free hemolysates were purified prior to globin preparation the α to β ratio did not change. On the other hand, globin extracted from whole iron-deficient cells, including the stroma, had a higher α to β ratio, 0.88 ± 0.04, but still significantly lower than normal. These results suggest that in iron deficiency there is a decrease in α-chain synthesis relative to β-chain and that there are membrane-bound globin chains, but no excessive increase in the free α-chain pool. Similar findings have been reported previously in other states of heme deficiency like sideroblastic anemia and lead poisoning.

scholarly journals Asynchronous globin chain synthesis in rabbit reticulocyte lystates induced by hemin-controlled repressor

Blood ◽  
1978 ◽  
Vol 51 (4) ◽  
pp. 653-658
Author(s):  
RS Franco ◽  
JW Hogg ◽  
OJ Martelo
Keyword(s):  
Globin Chain ◽  
Free System ◽  
Globin Chains ◽  
Reticulocyte Lysate ◽  
Chain Imbalance ◽  
Globin Synthesis ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

To define further the role of hemin-controlled repressor (HCR) in globin synthesis, we studied its effect on the synthesis of individual globin chains in a rabbit reticulocyte lysate cell-free system. In the presence of HCR there was a marked globin chain imbalance, resulting in a lowered alpha/beta ratio. These findings in vitro may have relevance to certain clinical heme deficiency states in which a similar globin chain imbalance has been observed.

Translation of Honeybee Promelittin Messenger RNA. Formation of a Larger Product in a Mammalian Cell-Free System

1975 ◽  
Vol 60 (1) ◽  
pp. 309-315 ◽  
Author(s):  
Gerda SUCHANEK ◽  
Ingela KINDAS-MUGGE ◽  
Gunther KREIL ◽  
Max H. SCHREIER
Keyword(s):  
Mammalian Cell ◽  
Messenger Rna ◽  
Free System ◽  
Cell Free System ◽  
Rna Formation

In vitroTranslation of Natural mRNAs in a Cell-free System Containing Components from Interferon-treated Chicken Fibroblasts and Factor Preparations from Mouse Ascites Cells or Rabbit Reticulocytes

1975 ◽  
Vol 30 (5-6) ◽  
pp. 398-405 ◽  
Author(s):  
G. Hiller ◽  
G. Viehhauser ◽  
I. Winkler ◽  
D. Pohl ◽  
C. Jungwirth ◽  
...  
Keyword(s):  
Chick Embryo ◽  
In Vitro Translation ◽  
Interferon Treatment ◽  
Mixed Cell ◽  
Free System ◽  
Free Protein ◽  
Cell Free System ◽  
Mouse Ascites ◽  
Rabbit Reticulocytes

Abstract Interferon Mechanism, in vitro Translation, Viral and Cellular mRNA, Chick Embryo Fibroblasts, Vaccinia Infection The effect of interferon has been studied in a mixed cell-free protein synthesizing system. Hemo­ globin (Hb) and Encephalomyocarditis virus (EMC) -RNA can be efficiently translated in vitro in a system containing S-30 lysates or run-off ribosomes from primary chick embryo fibroblasts (CEF) and a postmicrosomal supernatant from mouse ascites cells or a ribosomal-wash preparation from rabbit reticulocytes. Ribosomes prepared from CEF pretreated with high doses of homologous inter­ feron (500 units/ml) were able to translate Hb-RNA in the presence of heterologous factors with the same efficiency as ribosomes prepared from control cells. Translation of EMC-RNA was slightly reduced if ribosomes from interferon-treated cells were used in the mixed cell-free system, confirming previous reports. No inhibitory effect caused by interferon treatment of CEF cells could be detected on in vitro translation of natural mRNAs if the cells had, in addition to interferon treatment, been infected with vaccinia virus. Possible reasons for the different observations made with our cell-free protein synthesizing system from CEF and with cell-free systems prepared from mouse cells are discussed.

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