Globin-Chain Synthesis in Hb H Disease: the Activity of Red Cell Precursors and Their mRNA

2015 ◽  
pp. 2-6
Author(s):  
C. Kirschmann ◽  
Z. Lupovitz ◽  
M. Steinherz ◽  
R. Zaizov
Keyword(s):  
Red Cell ◽  
Globin Chain ◽  
Hb H Disease ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

scholarly journals Globin Chain Synthesis in the Marrow and Reticulocytes of Beta Thalassemia, Hemoglobin H Disease, and Beta Delta Thalassemia

Blood ◽  
1972 ◽  
Vol 40 (1) ◽  
pp. 105-111 ◽  
Author(s):  
Mordechai Shchory ◽  
Bracha Ramot
Keyword(s):  
Bone Marrow ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Globin Chain ◽  
Thalassemia Intermedia ◽  
Thalassemia Minor ◽  
Order Of Magnitude ◽  
Hb H Disease ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

Abstract α, β, and γ globin chain synthesis in bone marrow and peripheral blood reticulocytes were studied in two patients with thalassemia major, two with thalassemia intermedia, one with thalassemia minor, one with Hb H disease, and one with homozygous βδ-thalassemia. Nine nonthalassemic patients served as controls. In thalassemia major, a marked imbalance of α- to β-chain synthesis was found in the bone marrow as well as in reticulocytes. The imbalance, however, was slightly more evident in the latter. In the patients with thalassemia intermedia and minor the α- to β-globin chain ratios in the reticulocytes were of the same order of magnitude, despite the marked clinical differences between thalassemia intermedia and minor. A balanced synthesis was found in the bone marrow of the patient with thalassemia minor. The bone marrow globin synthesis in thalassemia intermedia was not studied. Contrary to that in Hb H disease and βδ-thalassemia, the imbalance was more apparent in the bone marrow. In the latter, no evidence for imbalance was detected in the reticulocytes. These results point out the need for further studies on globin chain synthesis in the bone marrow and reticulocytes of patients With the various thalassemia syndromes and the effect of the free globin chain pool on those results.

57 POSTNATAL CHANGES IN GLOBIN CHAIN SYNTHESIS IN HB H DISEASE

1977 ◽  
Vol 11 (1) ◽  
pp. 61
Author(s):  
R. Zaizov ◽  
C. Kirschmann ◽  
Y. Matoth
Keyword(s):  
Globin Chain ◽  
Hb H Disease ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

scholarly journals Alterations of Globin Chain Synthesis and of Red Cell Membrane Proteins in Congenital Dyserythropoietic Anemia I and II

1982 ◽  
Vol 16 (12) ◽  
pp. 1016-1021 ◽  
Author(s):  
Nicole Alloisio ◽  
Paule Jaccoud ◽  
Evelyne Dorleac ◽  
Laurette Morle ◽  
Noel Philippe ◽  
...  
Keyword(s):  
Membrane Proteins ◽  
Cell Membrane ◽  
Red Cell ◽  
Globin Chain ◽  
Red Cell Membrane ◽  
Congenital Dyserythropoietic Anemia ◽  
Cell Membrane Proteins ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

Globin Chain Synthesis in Haemoglobin New York (β 113 Valine→Glutamic acid

1980 ◽  
Vol 46 (4) ◽  
pp. 557-564 ◽  
Author(s):  
D. Todd ◽  
Vivian Chan ◽  
Rose G. Schneider ◽  
Andree M. Dozy ◽  
Y. W. Kan ◽  
...  
Keyword(s):  
New York ◽  
Glutamic Acid ◽  
Globin Chain ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

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 Delta +-thalassemia in Sardinia

Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 341-345 ◽  
Author(s):  
M Pirastu ◽  
R Galanello ◽  
MA Melis ◽  
C Brancati ◽  
A Tagarelli ◽  
...  
Keyword(s):  
Restriction Endonuclease ◽  
Beta Thalassemia ◽  
Beta Globin ◽  
Globin Chain ◽  
New Type ◽  
Polymorphic Restriction ◽  
Chain Synthesis ◽  
Double Heterozygosity ◽  
Globin Chain Synthesis

Abstract We have defined a new type of delta-thalassemia in which beta-globin chain synthesis is incompletely suppressed. Homozygotes have unusually low HbA2 levels, and double heterozygosity for this delta-thalassemia gene and beta-thalassemia normalizes the HbA2 level. The delta- thalassemia occurs on a chromosome that is identifiable using polymorphic restriction endonuclease sites. We call this condition delta +-thalassemia, to distinguish it from the previously described delta 0-thalassemia syndromes in which no delta-globin chain synthesis occurs.

Globin Chain Synthesis in Sickle Cell Trait Under Conditions of Folate Antagonism

1974 ◽  
Vol 51 (4) ◽  
pp. 236-239 ◽  
Author(s):  
George R. Honig ◽  
George Mason ◽  
Loyda N. Vida
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Globin Chain ◽  
Chain Synthesis ◽  
Globin Chain Synthesis

Studies of Globin Chain Synthesis and Globin mRNA Content in a Patient Homozygous for Hemoglobin Lepore

Hemoglobin ◽  
1978 ◽  
Vol 2 (2) ◽  
pp. 117-128 ◽  
Author(s):  
B. G. Forget ◽  
C. Cavallesco ◽  
E. J. Benz ◽  
P. D. McClure ◽  
D. G. Hillman ◽  
...  
Keyword(s):  
Globin Chain ◽  
Globin Mrna ◽  
Mrna Content ◽  
Chain Synthesis ◽  
Globin Chain Synthesis
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