bluuebunny/crossref_metadata_2025_split huggingface dataset:
Receptors That Induce Erythroid Differentiation of Ba/F3 Cells: Structural Requirements and Effect on STAT5 Binding
Authors: Pless Miklos , Norga Koenraad , Carroll Martin , Heim Markus H. , D'Andrea Alan D. , Mathey-Prevot Bernard 
Published: October 2019

AbstractEctopic expression of the erythropoietin receptor (EpoR) in the interleukin-3 (IL-3)–dependent cell line Ba/F3 results in growth and partial erythroid differentiation in Epo. In contrast, introduction and activation of the interleukin-5 receptor (IL-5R) or of the granulocyte-macrophage colony-stimulating factor receptor (GM-CSFR) results in proliferation only. As this effect is specific to the EpoR, the role of its extracellular or cytoplasmic domain in differentiation was tested after construction of two chimeric receptors. One receptor contained the extracellular domain of EpoR fused to the endodomain of IL-3R β-chain (E/β), while the other contained the EpoR cytoplasmic region fused to the extracellular domain of GM-CSFR α-chain (GMER). Surprisingly, both receptors induced differentiation ruling out a strict specificity of the extracellular or cytoplasmic region of EpoR in this process. Instead the ability to signal differentiation correlated with structural features shared by the EpoR, GMER, and E/β receptors. Dimerization of all three receptors results in the pairing of two signal transducing chains in the cytoplasm, in contrast to the mitogenic receptors IL-3R, IL-5R, GM-CSFR, which assemble as αβ heterodimers. Two new chimeric receptors that fulfilled the structural requirement exemplified by EpoR, but lacked any part of EpoR, were designed to consolidate this model. They consisted of the ectodomains of the GMR-α and IL-5Rα, respectively, fused to the endodomain of IL-3R β-chain. Both receptors were as effective as EpoR in signaling differentiation in response to their cognate ligand. Another property of receptors fulfilling these structural requirements is that they cause a marked delay in signal transducers and activators of transcription 5 (STAT5) activation on ligand stimulation. Taken together our studies show that structural assembly of receptors dictates their potential to signal erythroid differentiation in Ba/F3 cells, that differentiation can take place in the absence of Epo and that a delay in STAT5 activation is highly predictive of this process.
Thiol-Disulfide Isomerization in Thrombospondin: Effects of Conformation and Protein Disulfide Isomerase
Authors: Huang Evelyn M. , Detwiler Thomas C. , Milev Youli , Essex David W. 
Published: October 2019

AbstractThiol-disulfide isomerization in thrombospondin may affect the function of this adhesive protein. Two assays were developed to analyze the determinants of thiol-disulfide exchange and to correlate this exchange with thrombospondin conformation. (1) A competitive immunoassay for the EDTA-conformation of thrombospondin was developed with monoclonal antibody D4.6. (2) The free thiol(s) in thrombospondin was labeled with [3H]N-ethylmaleimide (NEM) under various conditions (the presence or absence of calcium, temperature, and pH), and thrombin digests of the labeled protein were analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). Consistent with previous reports, thrombin digest fragments of 150, 120, 20, and 14 kD were observed, each with radioactivity under some condition, plus a 25-kD peptide that was not labeled. Sequence data for these fragments and comparisons of SDS-PAGE analyses under reducing and nonreducing conditions indicated that Cys974 was the free thiol. The appearance of thiol label in the 120-kD fragment was previously shown to be a consequence of thiol-disulfide exchange (J Biol Chem 265:17859,1990) and label was recovered in this peptide only under conditions (absence of calcium, 37°C and pH 8.4) that led to the appearance of the EDTA-conformation of thrombospondin. Additional evidence for the correlation of EDTA-conformation and thiol-disulfide exchange was the enhanced conversion of thrombospondin to its EDTA-conformation in the presence of protein disulfide isomerase and the inability of thrombospondin pretreated with NEM to attain the EDTA-conformation. Flow cytometry with antibody D4.6 revealed platelet-associated thrombospondin in the EDTA-conformation in the presence of calcium, suggesting that the EDTA-conformation is a physiological conformation that does not necessarily require EDTA.
Thymocyte Fas Expression Is Dysregulated in Myasthenia Gravis Patients With Anti-Acetylcholine Receptor Antibody
Authors: Moulian Nathalie , Bidault Jocelyne , Truffault Frédérique , Yamamoto Ana Maria , Levasseur Philippe , Berrih-Aknin Sonia 
Published: October 2019

AbstractMyasthenia gravis (MG) is a human autoimmune disease mediated by anti-acetylcholine receptor (AChR) antibodies. The thymus is probably the site where the autoimmune response is triggered and maintained. Recent reports have linked various autoimmune disease with defective Fas expression. We thus analyzed Fas expression in thymocytes and peripheral blood lymphocytes (PBL) from MG patients. The proportion of a thymocyte subpopulation with strong Fas expression (Fashi) was markedly enhanced in MG patients with anti-AChR antibodies (P < .0003, compared with controls). In this group of patients, the proportion of CD4+Fashi and CD4+CD8+Fashi thymocytes were significantly increased (P < .002 for both subsets). Fashi thymocytes were enriched in activated cells and showed intermediate CD3 expression. They were preferentially Vβ5.1-expressing cells, previously shown to be enriched in potentially autoreactive cells. The proliferative response of thymocytes from MG patients to peptides from the AChR was abolished after depletion of Fashi cells. Fashi thymocytes were sensitive to an agonistic anti-Fas antibody. In peripheral blood, Fashi lymphocytes proportion was not significantly modified in MG patients whatever their anti-AChR antibody titer, compared with controls. Altogether, these results indicate that Fashi thymocytes, which accumulate in MG patients with anti-AChR antibodies, could be involved in the autoimmune response that targets the AChR.
Abnormal Erythrocyte Endothelial Adherence in Hereditary Stomatocytosis
Authors: Smith Brian D. , Segel George B. 
Published: October 2019

AbstractHereditary stomatocytosis is a red cell membrane protein disorder, which results in hemolytic anemia. Some patients with hereditary stomatocytosis experience dyspnea, chest pain, and abdominal pain, particularly after splenectomy. These symptoms may represent vaso-occlusion secondary to adherence of an abnormal erythrocyte membrane to vascular endothelium. We studied three members of a family with varying clinical expression of hereditary stomatocytosis. Adherence of red cells to endothelium was quantified by measuring the shear force required to separate individual cells from endothelial monolayers using a micropipette technique. Two patients with symptoms of in situ thromboses had a higher percentage of adherent cells compared with their asymptomatic sibling and normal controls. Correlation between this in vitro phenomenon and the clinical course suggests that flow abnormalities in the microcirculation attributable to erythrocyte endothelial adherence may play an important pathogenetic role in the illness. When the proportion of adherent red cells was reduced by a chronic transfusion program in one patient and pentoxifyllin therapy in another, the vaso-occlusive complications were eliminated.
The Effect of ACTH in Periodic (Cyclic) Neutropenia
Authors: CHAIKEN BERNARD H. 
Published: October 2019

Abstract
A case of periodic neutropenia of twenty years’ duration is presented. ACTH was administered intravenously in an attempt to abort or prevent the neutropenic cycles. No effect in raising the total leukocyte or neutrophil count was noted, and the neutropenic cycles could not be prevented by the ACTH. A striking absence of fever, stomatitis, or other secondary manifestations occurred during the neutropenic periods treated with ACTH. The background for treating such conditions with hormones is discussed.
Blood Coagulation and the L. E. Cell Phenomenon
Authors: LEE STANLEY L. , SCHWARTZ LAWRENCE I. , PARISER SANFORD 
Published: October 2019

Abstract
A factor derived from blood platelets has been shown to be necessary for the activation of the L. E. cell factor. This L. E. cell cofactor may be identical with the platelet thromboplastic factor. The relation of these findings to occurrence of the L. E. cell phenomenon in vivo and to the occurrence of a coagulation defect in systemic L. E. has been discussed.
The Significance of Proplasmocytes and Atypical Plasma Cells in the Benign Lymphocytic Meningitis Syndrome
Authors: WOLF PETER 
Published: October 2019

Abstract
A nontuberculous, lymphocytic meningitis with a low C.S.F. sugar level may be confused with a tuberculous meningitis. Additional diagnostic help may be obtained from examination of the blood. The blood pictures of twelve patients with tuberculous meningitis were compared with those from ten patients with a nontuberculous, nonbacterial lymphocytic meningitis, four of whom had low C.S.F. sugar levels. These ten patients appeared clinically to be suffering from the same disease and only the serum complement fixation test for lymphocytic choriomeningitis showed them to be a heterogeneous group.
The main differences in the blood were (1) a uniformly low total white cell count in the benign meningitis group as opposed to a widely varying total white cell count in tuberculous meningitis; (2) a lower percentage count of polymorphs in the benign lymphocytic meningitis group; (3) the presence of atypical plasma cells in nine of the ten patients in the benign, lymphocytic meningitis group. Two of the patients with benign meningitis, who had repeatedly given negative Paul-Bunnell tests showed a blood picture indistinguishable from that of glandular fever.
Although these atypical plasma cells are not specific for lymphocytic choriomeningitis or for virus diseases in general, they were found in no case of tuberculous meningitis. The presence of these cells is therefore of considerable aid in excluding a diagnosis of tuberculous meningitis.
An Unidentified Reticuloendothelial Cell in Bone Marrow and Spleen
Authors: SAWITSKY ARTHUR , HYMAN GEORGE A. , HYMAN JULIAN B. 
Published: October 2019

Abstract
1. A cell is described in the bone marrow aspirates of two patients which has not previously been fully described. This is a large reticuloendothelial-like cell filled with blue-staining granules. This cell has been found in successive bone marrow films aspirated from sternum and iliac crest.
2. These cells were also found in a splenic aspiration from one of the patients and were compared to similar cells from a case summarized by Möschlin.
3. The case reports show striking clinical similarities in the two patients, i.e. hepatosplenomegaly in relatively symptom-free individuals. This suggests that a common syndrome is present.
4. Differential histochemical studies of the granular material in the cytoplasm are reported.
5. The significance and function of these unusual cells in the marrow is unknown.
Metabolic Interrelations between Gastric Intrinsic Hematopoietic Factor and Vitamin B12
Authors: GLASS GEORGE B. JERZY , LILLICK LOIS C. , BOYD LINN J. , GUNNIS CARRIE , CORTI LUCY GORMAN 
Published: October 2019

Abstract
The concentration of vitamin B12 in blood serum and its excretion in the urine were assayed by the E. coli mutant technic in two patients after total gastrectomy and by use of the more sensitive Euglena gracilis method in four cases of pernicious anemia in relapse and 1 case of well controlled nutritional macrocytic anemia.
Following parenteral administration of 100 µg. vitamin B12 intramuscularly to two patients with total gastrectomy, much higher blood levels were observed within the first few hours than those reported by others under similar circumstances in normals.
Following oral administration of vitamin B12 together with a potent intrinsic factor concentrate from hog stomach, a rise in blood levels of vitamin B12 was observed in patients with pernicious anemia.
The normal serum levels of vitamin B12 could not be maintained, however, for the entire duration of the oral treatment, in spite of the apparent hematologic and clinical remission. The urinary output of vitamin B12 was in all instances less than 0.5 per cent of the total dose of vitamin B12 ingested during the experimental period, in spite of an optimal or suboptimal hematopoietic response obtained. A similar situation existed in patients with total gastrectomy, as well as in the patient with controlled macrocytic nutritional anemia, when vitamin B12 was ingested alone or with intrinsic factor concentrate.
The intensity of the obtained hematopoietic response in patients with pernicious anemia indicates the absorption of much larger amounts of vitamin B12 from the intestine than is indicated by the total urinary output of vitamin B12; therefore the anchorage and retention of a large part of vitamin B12 in the storage depots of the body after its absorption from the intestine must occur.
A sharp increase in the urinary output of vitamin B12 was observed during or immediately following the hematopoietic response in all patients with pernicious anemia treated orally with vitamin B12 and intrinsic factor concentrate, i.e., coincident with or immediately following the reticulocyte peak in blood. Similar observation was reported by the authors in the previous series of investigations. Of many possible interpretations of this finding, the most plausible appears to be the metabolic release of free vitamin B12 from its complex binding in the body during hyperactivity of the hematopoietic organs and its escape through the kidneys.
Number and Distribution of Human Hemic Cells
Authors: OSGOOD EDWIN E. 
Published: October 2019

Abstract
1. Methods are described which make possible the calculation of the number and distribution of the hemic cells of the body.
2. The numbers of these cells in the various blood-forming organs, in the blood outside these areas, and in the total body are summarized in table 3, together with the ratios of the number in the blood to the number outside the hematopoietic organs for the standard 70 Kg. human male. Dividing these figures by 70 will give the values per Kg.
3. By similar methods the values for other age and sex groups in man and for other species may be calculated.
4. Leukocytes apparently are not primarily blood cells but are merely en route in the blood to the defense of body tissue generally. Only 1/40 to 1/400 of the total leukocytes outside the hematopoietic organs are present in the blood stream at any one time.
5. These somewhat surprising figures account for many previously made observations which were hitherto hard to explain.
Radioactive Sodium Chromate for the Study of Survival of Red Blood Cells
Authors: WEINSTEIN IRWIN M. , SPURLING CARROLL L. , KLEIN HERMAN , NECHELES THOMAS F. 
Published: October 2019

Abstract
Cr51 erythrocyte survival times are reported in a group of patients with a variety of abnormal hemoglobin syndromes. Marked decreases in survival time are demonstrated in pure sickle cell anemia. Shortened survival times are reported in one case each of hemoglobin C disease and sickle cell-hemoglobin C disease with compensated hemolysis. Normal survival times are reported in sickle cell trait and hemoglobin C trait.
Red cell life span as measured by the Cr51 technic agrees well with most published reports of survival times in these disorders in cases performed with the Ashby technic. The Cr51 method appears to be as useful in measuring the survival of erythrocytes containing abnormal hemoglobins as it has been shown to be in other hemolytic disorders as well as in normals. Its decided advantages are its simplicity, adaptability, and reliability.
Intraerythrocytic Crystals in a White Patient with Hemoglobin C in the Absence of Other Types of Hemoglobin
Authors: DIGGS L. W. , KRAUS A. P. , MORRISON D. B. , RUDNICKI R. P. T. 
Published: October 2019

Abstract
Tetragonal crystals of hemoglobin were observed in approximately 2 per cent of erythrocytes of a sixteen-year-old white male of Italian parentage, who had hemoglobin C in the absence of A, S, and F.
The clinical and hematological features of the patient described are similar to those with "pure" hemoglobin C disease reported by others. The essential features are: chronic anemia of a mild degree, congestive splenomegaly, evidences of increased red cell destruction, hyperplastic marrow with increased numbers of immature erythrocytes in the peripheral blood, target cells and increased erythrocyte resistance to hyptonic saline solutions.
Electrophoretic analysis of hemolysates of both parents, one brother and a maternal uncle revealed hemoglobin A and C. The hemolysate of one sister revealed only hemoglobin A. None of the relatives examined had hemoglobin S or F and none showed hematologic abnormalities.
The Effect of Pyridoxine Deficiency Induced by Desoxypyridoxine on Acute Lymphatic Leukemia of Adults
Authors: WEIR DAVID R. , MORNINGSTAR WILLIAM A. 
Published: October 2019

Abstract
1. Pyridoxine deficiency can be induced in human beings by giving large doses of desoxypyridoxine while maintaining the patients on a normal diet.
2. In four cases of acute lymphoid disease of adults some modification of the disease appears to have been accomplished by the induction of pyridoxine deficiency. In one case a brief clinical remission occurred which seemed almost complete.
3. Possible other effects of the deficiency were adrenal cortical degeneration and deposition of crystals in the convoluted tubules of the kidneys. The adrenal changes could well be due to postmortem degeneration.
4. Other toxic effects of the desoxypyridoxine therapy were not observed.
5. Further trials of this type of therapy appear to be justified.
Symposium: What Is Hemophilia?: On the Nature and Diagnosis of Hemophilia
Authors: QUICK ARMAND J. 
Published: October 2019

Abstract
The primary cause of the defective hemostasis in hemophilia is the lack of thromboplastinogen in the blood. This anomaly is congenital and hereditary. The mechanism whereby the concentration of thromboplastinogen is depressed in the blood is not known and no satisfactory explanation can be given why the defect manifests itself only in the male. The degree of the hypothromboplastinogenemia determines the intrinsic severity of the disease. The diagnosis is based on establishing the existence of a bleeding tendency from birth, in demonstrating a deficiency of thromboplastinogen in the blood, and in finding a typical hereditary pattern.
The Effects of Tyrosinase on Certain Components of the Blood Clotting System
Authors: KRUGELIS EDITH J. , SIZER IRWIN W. 
Published: October 2019

Abstract
The clotting time of human and rat plasma was found to increase markedly after exposure to mushroom tyrosinase. The main, but not exclusive, effect of tyrosinase on the clotting system seems to be on fibrinogen, since the inactivating effect of tyrosinase can be greatly reduced by the subsequent addition of excess fibrinogen just before the clotting time of the plasma is measured. No evidence was found for any major effect of tyrosinase on any of the other components of plasma considered with the possible exception of prothrombin. Purified prothrombin is susceptible while prothrombin in plasma or serum is refractory to tyrosinase.
Leukemia in Atomic Bomb Survivors
Authors: MOLONEY WILLIAM C. , LANGE ROBERT D. 
Published: October 2019

Abstract
1. The ten cases of leukemia occurring among 3480 atomic bomb survivors in this study present convincing evidence of the high incidence of leukemia in atomic bomb survivors.
2. In all but one case blood studies were carried out prior to the development of pathognomonic features of leukemia. One case of reticulum cell leukemia was encountered on routine examination in a preclinical stage but with primitive or blast cells in the peripheral blood smears.
3. Of the ten cases, seven were acute or subacute leukemia and three were chronic myelogenous leukemia.
4. Observations on the acute cases of leukemia were of limited value because of the long interval in most cases between routine blood studies and the development of leukemia. However, in one case, examination ten weeks before clinical onset revealed no hematologic or physical findings suggestive of leukemia.
5. In contrast to acute leukemia, the long preclinical stage in two cases of chronic myelogenous leukemia allowed repeated hematologic and biochemical studies which were of considerable interest.
6. In these cases the long latent period and prolonged preclinical course were similar to those reported in cases of chronic myelogenous leukemia occurring spontaneously and following exposure to x-ray and other forms of radioactivity.
7. In the preclinical stage, disturbances in the red cells and platelets as well as leukocytosis with immature neutrophilic granulocytes were noted. The consistent presence of increased numbers of basophils, many of them atypical, was an outstanding feature of the peripheral blood smears in early myelogenous leukemia.
8. In two cases of preclinical myelogenous leukemia, studies on the separated leukocytes of the peripheral blood gave extremely low values for alkaline phosphatase.
9. Certain features of chronic myelogenous leukemia following irradiation suggest that the disorder may be due to a loss of growth-promoting or regulating factors.
Cytochemical Changes in Lymph Nodes and Spleen of Rats after Total Body X-Radiation
Authors: ACKERMAN G. ADOLPH , BELLIOS NICHOLAS C. , KNOUFF RALPH A. , FRAJOLA WALTER J. 
Published: October 2019

Abstract
Marked cytochemical changes were observed in the cells of the lymph nodes and spleen of one hundred and twenty-seven albino rats observed over periods of two hours to thirty-two days following a single dose of 600 r total body x-radiations. The intensity of the cytochemical reactions for pentosenucleoprotein, protein-bound sulfhydryl groups, and alkaline phosphatase decreased within two hours after x-radiation. This decrease in the cytochemical reactions occurred very rapidly and preceded any discernible morphologic alteration. Lymphocytes that were extremely sensitive to x-ray exhibited in most instances irreversible cytochemical changes. The cells that survived the lethal effects of x-radiations exhibited an increase in intensity of the reactions for pentosenucleoprotein, protein-bound sulfhydryl groups, and alkaline phosphatase between twelve hours and four days following irradiation. The reactions for desoxypentosenucleoprotein and mucopolysaccharides were unaltered in the cells of the lymph nodes and spleen following x-radiation.
Centrifugal Subfractionation of Polymorphonuclear Leukocytes, Lymphocytes and Erythrocytes
Authors: AGRANOFF BERNARD W. , VALLEE BERT L. , WAUGH DAVID F. 
Published: October 2019

Abstract
The separations of polymorphonuclear leukocytes from lymphocytes and erythrocytes has been accomplished by employing a method which takes advantage of their density differentials. During a single centrifugations the lymphocytes accumulate at a plasma-albumin interface (albumin I, density = 1.065 to 1.085), the polymorphonuclear leukocytes accumulate at an albumin I-albumin II interface (albumin II, density = 1.094) and the erythrocytes pass to the base of the Wintrobe tube. The yields are over 90 per cent pure cell types. All layers are isosmotic with blood.