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Biomedical subjects

A Maniatis

Publications and source records attributed to A Maniatis.

8 recordsLinked to original sources
PubMed1979

Hemoglobin Crete (beta 129 ala leads to pro): a new high-affinity variant interacting with beta o -and delta beta o -thalassemia.

Hemoglobin Crete, beta129 (h7)ala leads to pro, is a new mutant hemoglobin (Hb) with high oxygen affinity that was discovered in a Greek family in various combinations with beta- and deltabeta-thalassemia. The propositus, who presented an unusual clinical picture of an "overcompensated" hemolytic state, with erythrocytosis, splenomegaly, abnormal red cell morphology, and marked erythroid hyperplasia, appeared doubly heterozygous for Hb Crete and deltabeta-thalassemia. His red cells contained 67% Hb Crete and 30% Hb F, and the combination of these two hemoglobins resulted in a blood P50O2 of 11.2 mm Hg. A brother with Hb Crete trait (38% Hb Crete, 56% Hb A, blood P50O2 23.0 mm Hg) did not have significant erythrocytosis. Purified Hb Crete was heat-unstable and exhibited a high oxygen affinity, and a normal Bohr effect. We postulate that the beta 129 proline substitution disrupts the H helix, perturbing nearby residues involved in alpha 1 beta 1 contact sites of the Hb tetramer.

Adult↗
PubMed1979

Fetal characteristics of erythrocytes in sickle cell anemia: an immunofluorescence study of individual cells.

In a group of disease states that includes sickle cell anemia (SS disease), two fetal erythrocyte markers, Hb F and i antigen, persist into adulthood. Using the technique of single-cell immunofluorescence, we determined the expression of l-i antigens and the presence of Hb F within populations of erythrocytes. Subjects tested included normal adults, normal newborns, patients with SS disease, and individuals with sickle cell trait. We classified erythrocytes reacting to anti-i as i cells and those reacting to anti-l as l cells, a terminology analogous to that used to identify erythrocytes containing increased amounts of Hb F as F cells. The expression of l and i antigens within populations of both normal and SS erythrocytes was found to be heterogeneous. The proporations of both i cells and l cells in all SS patients studied exceeded those found in normal adults, and an overall stronger-than-normal reactivity of individual SS cells to the two antibodies was observed. Proportions of F cells showed no correlation with proportions of i cells; and with double fluroescence staining for both Hb F and i, a significant proportion of each total SS red cell population was found to carry only one or the other marker. These findings confirm and clarify on a cellular level our previous demonstration, by means of quantitative hemagglutination, that there is increased expression of both l and i by whole populations of SS erythrocytes. In addition, we provide here new information on the expression of l and i within populations of normal human erythrocytes.

Adult↗
PubMed1977

Increased expression in erythrocytic Ii antigens in sickle cell disease and sickle cell trait.

The reactivity of human erythrocytes with anti-I and anti-i sera was estimated in 36 normal adults, 18 individuals with sickle cell anemia and 27 individuals with sickle cell trait, by quantitative hemagglutination with a sensitive autoanalyzer system. SS erythrocytes showed significantly increased I and i reactivity when compared to normal erythrocytes. AS erythrocytes also demonstrated some increase in Ii reactivity. No correlation was found between erythrocytic HbF content and i reactivity.

Adult↗
PubMedDate not supplied

Direct antiglobulin tests in narcotic addicted patients.

Direct Antiglobulin Tests (DAT) as well as a number of immunological parameters (immunoglobulin levels, latex fixation, VDRL) were studied in 168 narcotic-addicted patients. Of these, 94 were untreated narcotic addicts (Group I), 61 were methadone maintained patients (Group II), and 13 had completed successful detoxification with methadone and were drug-free (Group III). Weakly positive DAT were detected in 2.1 per cent of individuals in Group I, 18 per cent in Group II, and 15 per cent in Group III. The reactions were usually transient. A number of other immunological aberrations were also present in individuals of all three groups, but there was no correlation between these parameters and the positive direct antiglobulin reactions.

Adolescent↗