Globin Synthesis in Sideroblastic Anaemia I α AND β PEPTIDE CHAIN SYNTHESIS

White, Joseph; Brain, M. C.; Ali, M. A. M.

doi:10.1111/j.1365-2141.1971.tb07037.x

Cited by 85 publications

(17 citation statements)

References 32 publications

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“…There is good evidence that in iron deficiency anaemia both globin [21] and haem biosynthesis are disturbed [2,6]. Thus, various authors confirmed that in this condition the free erythrocyte protoporphyrin (EPP) is increased [8,14,16; Lyberatos et cl., unpublished data].…”

mentioning

confidence: 66%

Erythrocyte <i>δ</i>-Aminolaevulinic Acid Dehydratase, Urinary Porphyrins and Porphyrin Precursors in Iron Deficiency Anaemia

Chalevelakis¹,

Lyberatos²,

Manopoulos³

et al. 1977

Acta Haematol

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In 20 iron deficient patients and 21 normal controls the activity of the enzyme δ-ALA dehydratase of erythrocytes was assayed. In addition the urine porphyrins and porphyrin precursor excretions were measured. It was found that in sideropenic patients the erythrocyte δ-ALA dehydratase activity was almost constantly higher than in normals; the difference of the mean values being statistically significant (p < 0.005). A significant diminution of δ-ALA (p < 0.0025) urine excretion was observed, whereas the urine excretion of PBG, CP and UP was found within the normal limits. The results are compared to those reported by other authors.

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mentioning

confidence: 66%

Erythrocyte <i>δ</i>-Aminolaevulinic Acid Dehydratase, Urinary Porphyrins and Porphyrin Precursors in Iron Deficiency Anaemia

Chalevelakis¹,

Lyberatos²,

Manopoulos³

et al. 1977

Acta Haematol

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“…Therefore, our observation of unbalanced synthesis with reduced non a / a ratio in CDA I and increased non a/a ratio in CDA I1 is at variance with the data available in the literature. Increased non a/a ratio has been recorded in sideroblastic anaemia (43). Although we did not study globin chain synthesis in bone marrow cells, it has been observed by other authors in various cases of CDA that unbalanced synthesis existed in precursor cells whenever it was recorded in peripheral blood (18,25,28,42).…”

Section: Discussionmentioning

confidence: 94%

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

et al. 1982

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Red cell membrane proteins were investigated in two unrelated children with congenital dyserythropoietic anemia (CDA) I and two siblings with CDA 11. The CDA I patients displayed globin chain synthesis imbalance, with reduction of the non a/cx ratio. One of the CDA I1 patients presented the reverse alteration. Whenever globin chain svnthesis was unbalanced. the membrane p-nitrophen~lphosphatask had an abnormally biphasic kinetics, consistent with substrate excess inhibition. as is observed in a-or P-thalassemic syndromes. One CDA 1 patient displayed a decrease of electrophoretic band 4.1 along with an ectopic phosphorylated protein at the level of band 4.2. In CDA I1 patients, band 3 was strikingly narrower than in controls. In CDA I1 and, to a lesser extent, in CDA I, the in vitro endogenous phosphorylation of band 2 + 2

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“…Imbalance in a/fl synthesis with a-chain excess has been reported in patients with certain types of sideroblastic anemia l (22).…”

Section: Discussionmentioning

confidence: 99%

Imbalance in α and β Globin Synthesis Associated with a Hemoglobinopathy

Rieder¹,

James²

1974

J. Clin. Invest.

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In contrast to findings in the thalassemia syndromes, studies of globin synthesis in subjects with structurally abnormal hemoglobins have generally revealed equal production of a and A polypeptide chains. However, in the present investigation of globin biosynthesis in vitro in blood and marrow from twoo subjects heterozygous for unstable hemoglobin Leiden, P66 or 7 Glu -* 0, a significant excess of a-chain production was revealed. A mother and daughter of northern European ancestry with mild compensated hemolytic anemia were found to have 25% hemoglobin Leiden. Increased hemolysis occurred after the ingestion of a sulfonamide and during infections. Normal levels of hemoglobin A2, 3.0 and 2.7%, and hemoglobin F, 0.8 and 0.6%, were found in the two subjects. Similar percentages of the minor hemoglobins were demonstrated in other family members without hemoglobin Leiden. After incubation of peripheral blood with [3H]-leucine, the #A/#Leiden synthesis ratio was 1.3, and the specific activity of pLelden was 1.3-2 times GAi. These results indicate preferential destruction of the unstable hemoglobin Leiden. However, in contrast to previous studies of other unstable hemoglobins, there was excess synthesis of a-chains. The total P/a synthesis ratio was 0.47-0.63 in peripheral blood and 0.82 in marrow. A pool of free a-chains was demonstrated by starch gel electrophoresis and DEAE column chromatography.

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Globin Synthesis in Sideroblastic Anaemia I α AND β PEPTIDE CHAIN SYNTHESIS

Cited by 85 publications

References 32 publications

Erythrocyte <i>δ</i>-Aminolaevulinic Acid Dehydratase, Urinary Porphyrins and Porphyrin Precursors in Iron Deficiency Anaemia

Erythrocyte <i>δ</i>-Aminolaevulinic Acid Dehydratase, Urinary Porphyrins and Porphyrin Precursors in Iron Deficiency Anaemia

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

Imbalance in α and β Globin Synthesis Associated with a Hemoglobinopathy

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