bluuebunny/crossref_metadata_2025_split huggingface dataset:
Characteristics and expression of binding sites specific for ferritin H- chain on human cell lines
Authors: Fargion S , Arosio P , Fracanzani AL , Cislaghi V , Levi S , Cozzi A , Piperno A , Fiorelli G 
Published: October 2019

Abstract
Purified recombinant human ferritin composed solely of H subunit was radiolabeled and incubated with proerythroleukemic K562 human cells. A specific binding was detected, and it could be displaced only by ferritins, natural or recombinant, containing large proportion of the H subunit. The specific ferritin H-chain binding was saturable, and cells showed 17,000 to 23,000 binding sites per cell. The affinity constant measured at 37 degrees C was of 3 x 10(8) M-1. Treatment with pronase eliminated the specific binding. The binding sites were expressed in a high number during the cellular exponential phase of growth and progressively decreased to disappear when cells reached the plateau phase. Treatment of the cells with desferrioxamine increased recombinant H-ferritin binding, while iron had little effect. K562 cells induced to differentiate by hemin failed to bind ferritin H. Ferritin H-chain binding capacity is present on various cell lines such as HL60, lung cancer, and hepatoma cells. Analysis of the binding sites by western blotting showed a peptide with apparent mol wt of about 100 kd.
Restoration of adenylate cyclase responsiveness in murine myeloid leukemia permits inhibition of proliferation by hormone. Butyrate augments catalytic activity of adenylate cyclase
Authors: Inhorn L , Fleming JW , Klingberg D , Gabig TG , Boswell HS 
Published: October 2019

Abstract
Mechanisms of leukemic cell clonal dominance may include aberrations of transmembrane signaling. In particular, neoplastic transformation has been associated with reduced capacity for hormone-stimulated adenylate cyclase activity. In the present study, prostaglandin E, a hormonal activator of adenylate cyclase that has antiproliferative activity in myeloid cells, and cholera toxin, an adenylate cyclase agonist that functions at a postreceptor site by activating the adenylate cyclase stimulatory GTP-binding protein (Gs), were studied for antiproliferative activity in two murine myeloid cell lines. FDC-P1, an interleukin 3 (IL 3)-dependent myeloid cell line and a tumorigenic IL 3- independent subline, FI, were resistant to these antiproliferative agents. The in vitro ability of the “differentiation” agent, sodium butyrate, to reverse their resistance to adenylate cyclase agonists was studied. The antiproliferative action of butyrate involved augmentation of transmembrane adenylate cyclase activity. Increased adenylate cyclase catalyst activity was the primary alteration of this transmembrane signaling group leading to the functional inhibitory effects on leukemia cells, although alterations in regulatory G- proteins appear to play a secondary role.
Lack of vimentin occurring during the intrafollicular stages of B cell development characterizes follicular center cell lymphomas
Authors: Moller P , Momburg F , Hofmann WJ , Matthaei-Maurer DU 
Published: October 2019

Abstract
Reactive germinal-center B cells completely lack vimentin. This observation made by using monoclonal vimentin antibody V9 and a sensitive immunoperoxidase technique led to the investigation of vimentin expression in an unselected series of 73 immunohistochemically proven B cell lymphomas. While small lymphocytic lymphomas regularly expressed vimentin, the neoplastic population of mixed, small and large, and large cell lymphomas often contained various proportions of vimentin-negative tumor cells or failed to express it at all. Against the background of the Lukes-Collins classification and the Kiel classification, both defining and subdividing follicular center-cell lymphomas, there were statistically highly significant correlations between the absence of vimentin and the histology, suggesting a germinal center origin. Our data suggest that loss of vimentin expression is indicative of the transient, follicular center-cell stage of normal and malignant B lymphocytic transformation. The complete absence of vimentin within the neoplastic population of all Burkitt's lymphoma cases examined may serve as an argument for its follicular center stage of differentiation, as was asserted by Lukes and Collins.
Spectrin Oran (alpha II/21), a new spectrin variant concerning the alpha II domain and causing severe elliptocytosis in the homozygous state
Authors: Alloisio N , Morle L , Pothier B , Roux AF , Marechal J , Ducluzeau MT , Benhadji- Zouaoui Z , Delaunay J 
Published: October 2019

Abstract
We report on spectrin Oran (alpha II/21), a new spectrin variant found in an Algerian family. It was characterized by the absence of the spots that classically correspond to the alpha II domain using two- dimensional analysis of spectrin limit digests. On the contrary, the abnormal domain was represented by a new set of spots in the 21-Kd and 16-Kd regions, as demonstrated by Western blots using anti-alpha II domain polyclonal antibodies. Spectrin Oran (alpha II/21) was found in the homozygous state in two children belonging to two separate branches of the family. It yields a severe elliptocytosis. Spectrin self- association was altered. The variant was much more difficult to prove in the heterozygous state, in which it results in no clinical and virtually no morphological symptom. In all four parents involved, however, electrophoretic analysis and Western blots showed the existence of the alpha II 21-Kd and 16-Kd peptides. In one parent, who combines spectrin Oran (alpha II/21) and the alpha II type-2 polymorphism, the two-dimensional spots (52, 39, 34, and 29 Kd) were quantified and appeared reduced by 30%: there was an intermediary decrease of spectrin self-association in this person. In the three other parents, spectrin Oran combined with the alpha II type-1 polymorphism. The alpha II type-1 spots (46, 35, 30, and 25 Kd) appeared in normal range, and spectrin self-association was normal. Along with previous observations, the present data emphasize the large fluctuations of the alpha-variant percentage. Provided spectrin Oran was present in a sufficient proportion, we found an associated alteration of the beta II domain (that faces the alpha II domain in the spectrin dimer): the beta II 65-Kd fragment was reduced and the beta II 52-Kd fragment was reciprocally increased.
Idiotype as a tumor-specific marker in childhood B cell acute lymphoblastic leukemia
Authors: Carroll WL , Link MP , Cleary ML , Bologna S , Carswell C , Amylon MD , Smith SD , Levy R 
Published: October 2019

Abstract
Immunoglobulin (Ig) or idiotype (Id) is a tumor-specific target in those B cell malignancies that express this molecule on their surface. We explored the biology of B cell acute lymphoblastic leukemia (B cell ALL) using Id as a tumor marker. In this report we describe the development of anti-Id monoclonal antibodies (MAB) for two children with B cell ALL. These reagents were used retrospectively to study tumor kinetics and to detect residual disease after chemotherapy. In both cases serum Id values were strikingly high at diagnosis (1.2 mg/mL and 10.8 mg/mL), suggesting that the tumor cells were relatively mature B cells capable of significant antibody production. In both patients the serum Id levels fell with the institution of therapy and confirmed that the patients were in remission. Increasing serum Id predicted relapse four months before conventional methods in patient 1, and Id proved to be a more sensitive measure of tumor burden than Southern blot analysis of rearranged Ig genes in bone marrow samples. Surprisingly, low levels of Id were redetected in the second patient just before completing therapy and have persisted for over a year despite the absence of clinical evidence of recurrent disease. Thus, serum Id levels reflect tumor burden during initial therapy but may not necessarily predict tumor progression after a complete clinical remission.
The population of paroxysmal nocturnal hemoglobinuria neutrophils deficient in decay-accelerating factor is also deficient in alkaline phosphatase
Authors: Burroughs SF , Devine DV , Browne G , Kaplan ME 
Published: October 2019

Abstract
In patients with paroxysmal nocturnal hemoglobinuria (PNH) the RBCs, neutrophils (PMNs), monocytes, and platelets derived from the abnormal clone are deficient in the complement-regulatory protein decay- accelerating factor (DAF). RBC acetylcholinesterase (AChE) and leukocyte alkaline phosphatase (LAP) activities are also characteristically low. DAF, AChE, and LAP are known to be anchored within cell membranes to glycophospholipid-containing phosphatidylinositol (PI).Because PNH progenitors contain DAF that appears to be lost with maturation, it has been proposed that this disorder results from abnormal tethering of these and possibly other proteins to membrane PI. We were puzzled, therefore, that our two PNH patients consistently had normal LAP levels. Consequently, we studied their isolated PMNs to compare DAF and LAP activities in individual cells.PMNs were separated by flow cytometry into DAF-positive and - negative populations by using rabbit anti-DAF antiserum and fluorescein- conjugated goat antirabbit IgG. In both patients the majority of PMNs were DAF deficient, and these cells contained very little alkaline phosphatase activity. In contrast, the smaller, DAF-positive cell populations were phosphatase replete. This is the first demonstration that abnormalities in DAF and LAP activity occur in the same PNH PMN population and strengthens the hypothesis that defective anchoring of proteins to membrane glycophospholipid underlies the pathophysiology of this disorder.
Functional dendritic cells are required for transfusion-induced sensitization in canine marrow graft recipients
Authors: Deeg HJ , Aprile J , Storb R , Graham TC , Hackman R , Appelbaum FR , Schuening F 
Published: October 2019

Abstract
Untransfused dogs given 9.2 Gy total-body irradiation and hematopoietic grafts from DLA-identical littermate donors uniformly achieve sustained engraftment, whereas dogs given three transfusions (Tx) of whole blood from the intended marrow donor 24, 17 and 10 days pretransplant uniformly reject their graft. Sensitization appears to be mediated by peripheral blood mononuclear cells and can be prevented by prior irradiation of the Tx product with UV light, known to inactivate leukocytes, in particular, cells with accessory function. In the present study we investigated which leukocyte population was responsible for Tx-induced sensitization and subsequent marrow graft rejection. Surprisingly, neither monocytes nor macrophages or dendritic cells induced sensitization, and all dogs so treated achieved engraftment; however, all four evaluable dogs transfused with UV- exposed blood to which small numbers of normal dendritic cells (12.5 x 10(3)/kg) were added rejected their marrow graft. Among five dogs given UV-exposed blood and normal monocytes (12.5 x 10(3)/kg) only one rejected its graft, and four achieved sustained engraftment. We conclude that donor dendritic cells are necessary, albeit not sufficient for in vivo sensitization. Sensitization is prevented by elimination or inactivation of dendritic cell.
Human immunodeficiency virus infection in patients with leukemia
Authors: Minamoto GY , Scheinberg DA , Dietz K , Gold JW , Chein N , Gee T , Reich LM , Hoffer J , Mayer K , Armstrong D 
Published: October 2019

Abstract
Eighteen human immunodeficiency virus (HIV)-seropositive patients were found among 211 previously treated adult patients with a variety of leukemias who had been multiply transfused before April 1985. Patients known to be homosexual or intravenous drug users were excluded from this study. The spouse of one HIV-seropositive patient became HIV infected and subsequently developed the acquired immune deficiency syndrome. Patients with leukemia who were multiply transfused before the availability of screening of blood products for HIV antibody should be counseled regarding their individual risks of HIV infection and the risk to sexual contacts.
A transient increase in histone H2A ubiquitination is coincident with the onset of erythroleukemic cell differentiation
Authors: Hensold JO , Swerdlow PS , Housman DE 
Published: October 2019

Abstract
Murine erythroleukemia cells are useful for studying the regulation of erythroid differentiation since these malignant pronormoblasts differentiate to orthochromatic normoblasts when treated with a variety of inducing agents. Changes in chromatin proteins have been described following inducer exposure. The significance of these changes, which are greatest in terminally differentiated cells remains unknown. Ubiquitin is a highly conserved 8.5 kilodalton peptide that is covalently linked to up to 10% of histone H2A. We demonstrate that following exposure of MEL cells to inducers of differentiation, a transient increase in ubiquitination of H2A occurs. This change is coincident with the onset of differentiation. This result suggests that ubiquitination of H2A may have a role in the nuclear changes necessary for erythroleukemic cell differentiation.
Low doses v conventional doses of corticoids in immune thrombocytopenic purpura (ITP): results of a randomized clinical trial in 160 children, 223 adults
Authors: Bellucci S , Charpak Y , Chastang C , Tobelem G 
Published: October 2019

Abstract
A randomized clinical trial was performed in 160 children and 223 adults with acute immune thrombocytopenic purpura (ITP). The role of corticoids at this phase of the disease is still controversial. Therefore, patients were randomized to receive either conventional doses (1 mg/kg/day) of corticotherapy or low doses (0.25 mg/kg/day) for 3 weeks. The remission, defined by platelet count superior to 100,000/microliter was studied for adults and children after 6 months and 12 months, respectively. The statistical analysis showed no significant difference between the two corticotherapy regimens neither in children nor in adults. Overall, 74% of children and 41% of adults were in remission. For the first time in acute ITP, a randomized prospective trial showed that both in children and adults low dose corticotherapy (0.25 mg/kg/day) proves to have the same efficacy as conventional doses (1 mg/kg/day).
Immunocytochemical localization of fibrinogen on washed human platelets. Lack of requirement for fibrinogen during adenosine diphosphate-induced responses and enhanced fibrinogen binding in a medium with low calcium levels
Authors: Suzuki H , Kinlough-Rathbone RL , Packham MA , Tanoue K , Yamazaki H , Mustard JF 
Published: October 2019

Abstract
The association of fibrinogen with washed human platelets was examined by immunocytochemistry during aggregation induced by adenosine diphosphate (ADP) and during deaggregation. The platelets were suspended either in a medium containing 2 mmol/L Ca2+ or in a medium containing no added Ca2+ (20 mumol/L Ca2+). Platelets were fixed at several times during aggregation and deaggregation, embedded in Lowicryl K4M, sectioned, incubated with goat antihuman fibrinogen, washed, reacted with gold-labeled antigoat IgG, and prepared for electron microscopy. To determine whether the method detected fibrinogen associated with the platelets, the platelets were pretreated with chymotrypsin (10 U/mL) and aggregated by fibrinogen; gold particles were apparent not only in the alpha granules but on the platelet surface and between adherent platelets as well. In the medium with 2 mmol/L Ca2+, ADP caused extensive aggregation of normal platelets in the presence of fibrinogen (0.4 mg/mL), and gold particles were evident between the adherent platelets and on the platelet surface; when the platelets deaggregated, gold was no longer present on the surface. In a medium without added Ca2+, ADP caused extensive aggregation in the presence of fibrinogen, and large numbers of gold particles were on the platelet surface and even more between adherent platelets. In this medium, the platelets did not deaggregate, and by five minutes, the granules appeared to be swollen or fused. In the absence of external fibrinogen, ADP caused the formation of small aggregates, and fibrinogen was not detected between adherent platelets. Thus, the association of fibrinogen with the platelet surface enhances platelet aggregation but is not essential for the ADP-induced formation of small aggregates. The association of fibrinogen with platelets is greater under conditions in which platelets release their granule contents and do not deaggregate because both endogenous and exogenous fibrinogen take part in aggregation.
A new procoagulant in acute leukemia
Authors: Falanga A , Alessio MG , Donati MB , Barbui T 
Published: October 2019

Abstract
To verify whether cancer procoagulant (CP), a cysteine proteinase procoagulant distinct from tissue factor (TF), is associated with leukemic cells, we assayed the procoagulant activity of blast cell extracts from 26 patients with different cytological subtypes of acute nonlymphoid leukemia (ANLL) according to the French-American-British classification. All the samples except two shortened the recalcification time of normal human plasma, the effect being significantly greater in the M3 subgroup. The two criteria used to distinguish between CP and TF, independence from factor VII in initiating blood coagulation and sensitivity to cysteine-proteinase inhibitors, were positive in 19 samples from M1, M2, M3, and M4 cytological subtypes. None of the M5 samples fulfilled these criteria. In addition, M1, M2, M3, and M4 samples immunoreacted with an anti-CP goat polyclonal antibody on an Ouchterlony immunodiffusion plate. This study provides the first evidence for a procoagulant other than TF that is associated with leukemic cells.
Excess heme in sickle erythrocyte inside-out membranes: possible role in thiol oxidation
Authors: Kuross SA , Rank BH , Hebbel RP 
Published: October 2019

Abstract
It has been suggested that the development of sickle RBC membrane defects might be related to abnormal amounts of membrane-associated heme (a term we use in its generic sense to include hemoglobins, hemichromes, and free heme). Techniques previously used to measure membrane heme, however, would not distinguish between what is truly membrane-associated and what is merely trapped in RBC ghost preparations. Consequently, we have examined extensively washed inside- out membranes (IOM) prepared from normal and sickle RBC. Approximately 25% of the sickle ghost heme is lost upon conversion to IOM, but sickle IOM still have a significant excess (1.6 +/- 0.3 nmol heme/mg membrane protein compared with 0.7 +/- 0.2 nmol/mg for normal IOM, P less than .001). Amounts of ghost heme are only poorly predictive of amounts of IOM heme (r = .664). Preparation of IOM by using isotonic lysis with saponin yields virtually identical amounts of IOM heme. Small amounts of heme (less than 15%) can be displaced from IOM by using manipulations that elute spectrin, displace electrostatically bound proteins, or cleave the cytoplasmic portion of band 3. Treatment of IOM with dithiothreitol (DTT), however, displaces the most heme (35%), and this is almost reproduced (25% displacement) by the treatment of intact RBC with DTT before IOM preparation. Sequential treatment with all manipulations still leaves about 40% of the heme in sickle IOM, which indicates a compartment more intimately associated with the membrane. At least part of this is free heme without globin, as evidenced by abnormal binding of radiochloroquine to sickle IOM. Conversely, some IOM-associated globin is globin without heme because the measurement of globin per se markedly overpredicts amount of IOM heme. There is a strong correlation between RBC density and amounts of either ghost or IOM heme. Finally, the amount of membrane thiol oxidation (as measured by thiol-disulfide-exchange chromatography) does not correlate at all with ghost heme (r = .105), but it correlates well with IOM heme (r = .877, P less than .001). These data demonstrate that there are abnormal amounts of heme truly associated with sickle RBC membranes, and they are consistent with the hypothesis that this membrane-associated heme participates in the pathobiology of the sickle RBC membrane, particularly those aspects perhaps related to thiol oxidation.
Thrombasthenia with an abnormal platelet membrane glycoprotein IIb of different molecular weight
Authors: Jung SM , Yoshida N , Aoki N , Tanoue K , Yamazaki H , Moroi M 
Published: October 2019

Abstract
We describe an individual with abnormal platelet glycoprotein (GP) IIb of different molecular weight (mol wt), a defect that distinguishes this patient from previously reported thrombasthenics. The patient, a 21-year-old female, has a mild bleeding tendency; her platelets lack adenosine diphosphate (ADP) aggregation and have severely suppressed collagen aggregation but a normal response to ristocetin. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis of her platelets indicates that they contain two types of GPIIb molecules: one with an abnormal mol wt (122 kd, unreduced; 128 kd, reduced) and one with a normal mol wt (128 kd, unreduced; 118 kd, reduced). Relative to the amount of GPIIb in normal platelets, her platelets contain approximately 35% abnormal GPIIb and 20% normal GPIIb. Fibrinogen binding assays on the patient's platelets indicated that they contained 25% of the normal amount of fibrinogen receptors. Crossed immunoelectrophoresis of the patient's platelets demonstrated the formation of a GPIIb/IIIa complex that was mainly composed of normal mol wt GPIIb and GPIIIa. The patient's father has decreased ADP aggregability, and his platelets also contained both abnormal and normal GPIIb (about 50% of the normal level and about 50% of the normal number of fibrinogen receptors); her mother has only normal GPIIb. These results indicate that the patient has heterozygous GPIIb molecules with an abnormality of GPIIb at the molecular level. Studies on this abnormal GPIIb should provide information about the function of GPIIb and the mechanism of its biosynthesis.
The human genes for GM-CSF and IL 3 are closely linked in tandem on chromosome 5
Authors: Yang YC , Kovacic S , Kriz R , Wolf S , Clark SC , Wellems TE , Nienhuis A , Epstein N 
Published: October 2019

Abstract
As demonstrated by long-range mapping of restriction endonuclease recognition sequences and genomic cloning, we found that the human genes encoding interleukin 3 (IL 3) and granulocyte/macrophage colony- stimulating factor (GM-CSF) are tandemly arrayed on the long arm of chromosome 5, separated by 9 kilobases (kb) of DNA. This close physical linkage of genes with similar structure and biologic function suggests that these cytokines may have evolved from a common ancestral gene. This linkage in evolution of two relatively divergent genes further implies that some of the other lymphokine and cytokine genes that appear to share as much or more sequence similarity than do IL 3 and GM- CSF may be distantly related members of a cytokine gene family.