scholarly journals Absolute Rates of Globin Chain Synthesis in Thalassemia

Blood ◽  
1968 ◽  
Vol 31 (2) ◽  
pp. 226-233 ◽  
Author(s):  
ARTHUR BANK ◽  
ALBERT S. BRAVERMAN ◽  
JOYCE V. O’DONNELL ◽  
PAUL A. MARKS
Keyword(s):  
Peripheral Blood ◽  
Erythroid Cell ◽  
Absolute Rate ◽  
Globin Chain ◽  
Globin Chains ◽  
The Absolute ◽  
Α Chain ◽  
Chain Synthesis ◽  
Globin Chain Synthesis ◽  
Β Chain

Abstract The absolute rate of α chain synthesis per erythroid cell in the peripheral blood of patients with β thalassemia has been shown to be normal while that of β chains is markedly decreased or absent. The results indicate that α chains do not require the presence of β chains for their normal synthesis and release. In addition, γ chain synthesis does not compensate for the decreased β chain synthesis. A marked heterogeneity in the amount of β globin chains produced by different patients with β thalassemia is also prominent.

scholarly journals Globin chain synthesis in HbD (Punjab)-beta-thalassemia

Blood ◽  
1976 ◽  
Vol 47 (1) ◽  
pp. 113-120 ◽  
Author(s):  
RF Rieder
Keyword(s):  
Bone Marrow ◽  
Peripheral Blood ◽  
Beta Thalassemia ◽  
Globin Chain ◽  
Globin Chains ◽  
Mild Anemia ◽  
Thalassemia Trait ◽  
Chain Synthesis ◽  
Hbd Punjab ◽  
Globin Chain Synthesis

Abstract A 23-yr-old man of Greek-Italian ancestry with mild anemia was found to be heterozygous for HbD (Punjab) beta121 glu leads to gin and beta- thalassemia. HbA was not detected upon electrophoresis of the subject's hemolysate, and no synthesis of betaA globin was demonstrated after incubation of peripheral blood or bone marrow with 3H-leucine. The thalassemia gene was thus of the betao variety. The betaD/alpha synthesis ratios were almost equally unbalanced in the blood and bone marrow: 0.53 and 0.61, respectively. The mother of the propositus had beta-thalassemia trait. In peripheral blood the betaA/alpha synthesis ratio was 0.38. The mutant betaD gene thus appeared potentially capable of directing the synthesis of globin chains as efficiently as a normal betaA gene. The mildness of the HbD-betao-thalassemia syndrome appeared to be due to the maintenance of a relatively high total beta/alpha synthesis ratio in the presence of a physiologically neutral structural mutation.

scholarly journals Globin chain synthesis in HbD (Punjab)-beta-thalassemia

Blood ◽  
1976 ◽  
Vol 47 (1) ◽  
pp. 113-120
Author(s):  
RF Rieder
Keyword(s):  
Bone Marrow ◽  
Peripheral Blood ◽  
Beta Thalassemia ◽  
Globin Chain ◽  
Globin Chains ◽  
Mild Anemia ◽  
Thalassemia Trait ◽  
Chain Synthesis ◽  
Hbd Punjab ◽  
Globin Chain Synthesis

A 23-yr-old man of Greek-Italian ancestry with mild anemia was found to be heterozygous for HbD (Punjab) beta121 glu leads to gin and beta- thalassemia. HbA was not detected upon electrophoresis of the subject's hemolysate, and no synthesis of betaA globin was demonstrated after incubation of peripheral blood or bone marrow with 3H-leucine. The thalassemia gene was thus of the betao variety. The betaD/alpha synthesis ratios were almost equally unbalanced in the blood and bone marrow: 0.53 and 0.61, respectively. The mother of the propositus had beta-thalassemia trait. In peripheral blood the betaA/alpha synthesis ratio was 0.38. The mutant betaD gene thus appeared potentially capable of directing the synthesis of globin chains as efficiently as a normal betaA gene. The mildness of the HbD-betao-thalassemia syndrome appeared to be due to the maintenance of a relatively high total beta/alpha synthesis ratio in the presence of a physiologically neutral structural mutation.

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 Imbalanced globin chain synthesis determines erythroid cell pathology in thalassemic mice

Haematologica ◽  
2009 ◽  
Vol 94 (9) ◽  
pp. 1211-1219 ◽  
Author(s):  
K. Srinoun ◽  
S. Svasti ◽  
W. Chumworathayee ◽  
J. Vadolas ◽  
P. Vattanaviboon ◽  
...  
Keyword(s):  
Erythroid Cell ◽  
Globin Chain ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

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.

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