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Biomedical subjects

E Beutler

Publications and source records attributed to E Beutler.

At least 361 records · Page 20Linked to original sources
PubMed1980

Plasma blood group glycosyltransferase activities after bone marrow transplantation.

Human blood groups (ABO) are known to be determined by the terminal glycosyl residues attached to common carbohydrate chains of the red cell surface. N-acetylgalactosaminyltransferase (A-enzyme) in blood group A persons and galactosyltransferase (B-enzyme) in blood group B persons are responsible for producing A and B substances on the red cell surface, with both enzymes absent in blood group O persons. The plasma transferase (A - and B-) activity was assayed after the complete replacement of the bone marrow of patients with acute leukemia or aplastic anemia by transplantation bone marrow from donors with ABO blood group differing from the recipient. The patient's blood type completely changed from the recipient's type to the donor's type. However, the A- and B-enzyme activities of the patients changed only slightly after bone marrow transplantation. The results indicate that most of the A- and B-enzymes in the circulatory plasma is not derived from the bone marrow, lymphoid, or macrophage tissue. Other tissues must be the primary source of the enzymes in plasma.

ABO Blood-Group System↗
PubMed1980

Genetic markers in human bone marrow transplantation.

Blood cell isozymes, red cell antigens, immunoglobulin allotypes, and marker chromosomes are suitable tools to monitor bone marrow engraftment and marrow graft quality. Data on genetic markers from 26 patients who underwent bone marrow transplantation as a treatment for acute leukemia are presented here.

Blood Group Antigens↗
PubMed1980

Glucose-6-phosphate dehydrogenase variants: reexamination of G6PD Chicago and Cornell and a new variant (G6PD Pea Ridge) resembling G6PD Chicago.

Two large and unrelated families were investigated for hereditary nonspherocytic hemolytic anemia associated with deficiency of erythrocyte glucose-6-phosphate dehydrogenase (G6PD). In both families, the kinetic and electrophoretic features of the G6PD variants resembled those of G6PD Chicago. Further investigation revealed that members of one of these families previously had been characterized as having the G6PD variants Chicago and Cornell. However, it is clear that each of these terms has been applied to the same variant in this single large kindred. In the second family, we describe a newly identified variant with unique characteristics, which we have designated G6PD Pea Ridge. G6PD Pea Ridge resembles G6PD Chicago but differs in electrophoretic mobility and in a few kinetic parameters. It exhibits an unusually high Ki for NADPH and thus appears to be insensitive to product inhibition. As other cases previously considered to be the Chicago variant become more fully characterized, this probably will be shown to be a heterogeneous group of variants.

Adult↗
PubMed1980

The Woronets trait: a new familial erythrocyte anomaly.

A dominantly inherited abnormality of the red blood cells is described. It is characterized by the presence of a small population of markedly distorted red blood cells resembling keratocytes. In addition, many of the red cells show minor deformities resembling the beginning stages of formation of echinocytes. Red cell lifespan is normal, and the severely deformed cells appear to represent the senescent population. Inappropriate increases in activities of some, but not all, age-dependent red cell enzymes were observed. The anomaly appeared to be entirely benign; no abnormal clinical findings were associated with this defect. It has been designated the Woronets trait.

Adolescent↗
PubMed1980

Red cell glycolytic intermediates in diabetic patients.

The glycolytic intermediates of the red cells of 11 adult diabetic patients were compared with those of eight normal controls. A statistically significant elevation of glucose-6-P and fructose-6-P levels were correlated with the blood sugar levels and 2,3-DPG levels. The absence of a metabolic crossover point suggests that the effect of high levels of glucose may be exerted through regulation of the hexokinase step, itself, but the nature of the regulation cannot be deduced from the complex situation which exists in vivo.

Aged↗
PubMed1980

Glycosylated hemoglobin A2 components.

Partially purified hemoglobin A2 has been examined for the existence of glycosylated components by isoelectric focusing and by acid agar gel electrophoresis. Bands analogous to the glycohemoglobin derivatives of hemoglobin A, hemoglobin-A1.a.b.c, were readily detected. Evidence that these minor bands are in fact glycohemoglobins was obtained by showing that 14C-glucose bound to hemoglobin A2 moved with these minor bands. The amounts of glycohemoglobin derivatives of hemoglobin A2 were increased in the blood of diabetic patients.

Diabetes Mellitus↗
PubMed1980

Gaucher's disease.

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Europe, Eastern↗
PubMed1979

Three new variants of glucose-6-phosphate dehydrogenase associated with chronic nonspherocytic hemolytic anemia: G-6-PD Lincoln Park, G-6-PD Arlington Heights, and G-6-PD West Town.

Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency was identified in three children who were evaluated because of chronic nonspherocytic hemolytic anemia. One child is of German extraction, another Puerto Rican, and the third Mexican. In each of the patients the hemolytic process was well compensated, but each had one or more episodes of anemia following exposure to an oxidant drug or with infections. The electrophoretic, functional, and kinetic properties of the mutant enzymes, derived both from the patients' erythrocytes and from cultured fibroblasts, allowed each to be distinguished from G-6-PD variants previously described.

Anemia, Hemolytic, Congenital Nonspherocytic↗
PubMed1979

Incorporation of glucocerebrosidase into Gaucher's disease monocytes in vitro.

Several carriers were evaluated for use in the delivery of exogenous glucocerebrosidase to monocytes from Gaucher's disease patients. Only gamma globulin-coated, resealed erythrocytes proved to be an effective vehicle for enzyme delivery. Glucocerebrosidase added in this manner normalized intracellular enzyme levels for at least 18 hr. In this model system for the study of enzyme replacement therapy, soluble enzyme, enzyme in uncoated resealed erythrocytes, and enzyme incorporated into liposomes were ineffective.

Cells, Cultured↗
PubMed1979

Comparison of structure and function of human erythrocyte and human muscle actin.

Human erythrocyte actin and human skeletal muscle actin were purified by acetone powder extraction and gel filtration. Pure human erythrocyte actin resembles muscle actin in its polymerization and depolymerization by phalloidin, cytochalasin B, and DNase I, in its peptide mapping pattern, and in the amino acid composition of corresponding peptides. Isoelectric focusing gel analysis showed that human erythrocyte actin exists in the beta/gamma form, but muscle actin is in the alpha form. Abnormal deformability of resealed erythrocyte membranes was observed after incorporation of the actin-specific agents, phalloidin and DNase I, suggesting that erythrocyte actin might function as a membrane structural element to maintain erythrocyte membrane deformability.

Actins↗
PubMed1979

Terminal transferase in leukemia of adults.

Terminal deoxynucleotidyl transferase (TdT) determinations were carried out on peripheral blood leukocytes or bone marrow cells from 61 adult patients with various types of leukemia. TdT activity was undetectable in the cells of patients with acute myelocytic or acute myelomonocytic leukemia but was present in 12 of 13 patients with acute nonmyelocytic leukemia. None of 3 patients with acute myelocytic transformation of chronic myelocytic leukemia (CML) manifested TdT activity while 4 of 6 patients with lymphoid transformation had such activity. More patients with TdT in their leukemic cells responded to treatment than those without TdT activity. However, our findings suggest that TdT activity may be less useful in management of leukemia than has sometimes been supposed.

Acute Disease↗