Abstract:
:The alpha thalassemias are associated with a decrease in alpha chain synthesis. Hemoglobin H (HbH) disease is a moderately severe form of alpha thalassemia characterized by the production of 5%--20% of HbH, while alpha thalassemia trait is a milder form of alpha thalassemia. In two patients with HbH disease, the ratio of alpha chain synthesis to beta chain synthesis (alpha/beta ratio) was decreased in both bone marrow cells and reticulocytes. When isolated mRNA from bone marrow cells and reticulocytes was translated in a heterologous cell-free system, the alpha/beta ratios were lower than the intact cell ratios. These findings were confirmed by hybridization of the mRNA of both marrow cells and reticulocytes using purified alpha and beta cDNA probes. In the intact cells of two patients with alpha thalassemia trait, the alpha/beta ratios were also decreased and were similar in marrow cells and reticulocytes. Cell-free studies of translatable mRNA also demonstrated decreased alpha/beta ratios, but, unlike the HbH studies, the cell-free alpha/beta ratios were similar to the intact cell ratios. One hybridization study utilizing peripheral blood mRNA had an alpha/beta ratio consistent with the cell-free ratios. These results indicated that, in both HbH disease and alpha thalassemia trait, there was decreased alpha globin mRNA present in both nucleated red cell precursors and reticulocytes. In addition, the data suggested that there may be translational mechanisms that operate in intact HbH cells which attempt to balance globin chain production. In alpha thalassemia trait cells, no such controls appeared to be active and globin chain synthesis was directly proportional to the amount of alpha and beta globin mRNA in the cells.
journal_name
Bloodjournal_title
Bloodauthors
Natta CL,Ramirez F,Wolff JA,Bank Asubject
Has Abstractpub_date
1976-06-01 00:00:00pages
899-907issue
6eissn
0006-4971issn
1528-0020journal_volume
47pub_type
杂志文章相关文献
BLOOD文献大全abstract::Type I interferon (IFNalpha/beta) plays a complex role in HIV-1 infection and has been proposed alternately to have roles in either disease protection or progression. Although IFNalpha/beta plays crucial roles in regulating monocytes and dendritic cells, responsiveness of these cells to IFNalpha/beta in HIV-1 infectio...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-11-190231
更新日期:2009-05-28 00:00:00
abstract::Recombinant erythropoietin (EPO) and iron substitution are a standard of care for treatment of anemias associated with chronic inflammation, including anemia of chronic kidney disease. A black box warning for EPO therapy and concerns about negative side effects related to high-dose iron supplementation as well as the ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2019004653
更新日期:2020-08-27 00:00:00
abstract::Somatic hypermutation and class-switch recombination of the immunoglobulin (Ig) genes occur in germinal center (GC) B cells and are initiated through deamination of cytidine to uracil by activation-induced cytidine deaminase (AID). Resulting uracil-guanine mismatches are processed by uracil DNA glycosylase (UNG)-media...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2015-02-628164
更新日期:2016-01-07 00:00:00
abstract::Besides regulating leukocyte trafficking in normal and injured tissues, several chemokines may positively or negatively regulate angiogenesis. Here we report that CCL16 activates an angiogenic program in vascular endothelial cells by activating CCR1. CCL16 induces dose-dependent random and directional migration of end...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2003-05-1387
更新日期:2004-01-01 00:00:00
abstract::We have generated a large, unique database that includes morphologic, clinical, cytogenetic, and follow-up data from 2124 patients with myelodysplastic syndromes (MDSs) at 4 institutions in Austria and 4 in Germany. Cytogenetic analyses were successfully performed in 2072 (97.6%) patients, revealing clonal abnormaliti...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2007-03-082404
更新日期:2007-12-15 00:00:00
abstract::The molecular basis of different outcomes in pediatric acute lymphoblastic leukemia (ALL) remains poorly understood. We addressed the clinical significance and mechanisms behind in vitro cellular responses to ionizing radiation (IR)-induced DNA double-strand breaks in 74 pediatric patients with ALL. We found an apopto...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-03-142950
更新日期:2009-01-01 00:00:00
abstract::Surface receptors specific for either the C4b (CR1) or C3d (CR2) component of complement were examined on the neoplastic cells from 30 cases of non-Hodgkin's lymphoma of B-cell origin and on cells derived from 9 normal lymphoid tissues. Lymphocyte suspensions from non-neoplastic peripheral blood, tonsils, and lymph no...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1981-07-01 00:00:00
abstract:UNLABELLED:Polymorphonuclear neutrophil granulocytes (neutrophils) are tightly controlled by an incompletely understood homeostatic feedback loop adjusting the marrow's supply to peripheral needs. Although it has long been known that marrow cellularity is inversely correlated with G-CSF levels, the mechanism linking pe...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2012-05-429589
更新日期:2013-01-31 00:00:00
abstract::Evidence has been provided recently that shows that high concentrations of cytokines can fulfill functions previously attributed to stromal cells, such as promote the survival of, and led to a net increase in human primitive progenitors initiating long-term cultures in vitro (LTC-IC) or engrafting NOD-SCID (nonobese d...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1999-07-15 00:00:00
abstract::Approximately 20% of children with acute lymphoblastic leukemia (ALL) relapse because of failure to eradicate the disease. Current drug efficacy studies focus on reducing leukemia cell burden. However, if drugs have limited effects on leukemia-initiating cells (LICs), then these cells may expand and eventually cause r...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2012-08-448852
更新日期:2013-02-21 00:00:00
abstract::Proapoptotic Bax and Bak are the key B-cell lymphoma-2 family members mediating apoptosis through the intrinsic pathway. Cells doubly deficient for Bax and Bak are profoundly resistant to apoptotic stimuli originating from multiple stimuli. Here we describe mice in which Bax and Bak have been deleted specifically in T...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2010-04-279687
更新日期:2010-12-09 00:00:00
abstract::Tryptases are serine proteases primarily expressed in mast cells. Normal blood basophils express only trace amounts of the enzyme. However, recent immunohistochemical studies have raised the possibility that neoplastic basophils express significant amounts of tryptase. In this study, tryptase expression was analyzed i...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.v98.8.2580
更新日期:2001-10-15 00:00:00
abstract::The novel immunosuppressant FTY720 activates sphingosine 1-phosphate receptors (S1PRs) that affect responsiveness of lymphocytes to chemokines such as stromal cell-derived factor 1 (SDF-1), resulting in increased lymphocyte homing to secondary lymphoid organs. Since SDF-1 and its receptor CXCR4 are also involved in bo...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2003-03-0875
更新日期:2004-06-15 00:00:00
abstract::We have used immunogold staining to locate thrombospondin (TSP) on thrombin-activated human platelets, and have compared its distribution with that of fibrinogen (or fibrin) on thrombin- and ADP-stimulated platelets. To do this, isolated platelets were incubated with monospecific antibodies to TSP or fibrinogen (fib) ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1985-04-01 00:00:00
abstract::High concentrations of bovine factor VIII cause clumping of platelets into a few very large aggregates. This response is termed superaggregation. It is distinct from factor-VIII-induced agglutination but is also independent of both extracellular calcium ions and platelet energy metabolism. Neither agglutinating lectin...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1982-12-01 00:00:00
abstract::Endothelial cells play an important role in the regulation of fibrinolysis by the production of several key regulatory proteins. The cytokines tumor necrosis factor (TNF), lymphotoxin, and interleukin-1 (IL-1), but not interleukin-6, increase the production of plasminogen activator inhibitor-1 (PAI-1) by endothelial c...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1990-05-15 00:00:00
abstract::We tested the hypothesis that the antibody response to human factor IX in mice is controlled by genetic factors, especially histocompatibility antigens. Seven inbred mouse strains were immunized against human factor IX by adenoviral gene transfer or serial injections of human factor IX protein. A/J mice had the highes...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2004-03-1126
更新日期:2005-02-01 00:00:00
abstract::Primary central nervous system lymphoma (PCNSL) is confined to the brain, eyes, and cerebrospinal fluid without evidence of systemic spread. Rarely, PCNSL occurs in the context of immunosuppression, e.g. post-transplant lymphoproliferative disorders (PTLD) or HIV (AIDS-related PCNSL). These cases are poorly characteri...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2020008520
更新日期:2020-11-17 00:00:00
abstract::Receptor tyrosine kinases are known to be important in growth and differentiation. We have recently found that c-kit, the tyrosine kinase receptor for steel factor, also regulates cell-matrix adhesion. Because Steel factor helps regulate cell migration and localization, this may be an important biologic function. Inte...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1995-09-15 00:00:00
abstract::Previous studies on patients with storage pool deficiency (SPD) who are specifically deficient in platelet dense granules (delta-SPD) have suggested a role for dense granule substances, in all likelihood adenosine diphosphate (ADP), in mediating thrombus formation on subendothelium at high shear rates. The role of den...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1996-05-15 00:00:00
abstract::Adaptive immunity is triggered at the immune synapse, where peptide-major histocompatibility complexes and costimulatory molecules expressed by dendritic cells (DCs) are physically presented to T cells. Here we describe transmission of the inflammatory monoamine serotonin (5-hydroxytryptamine [5-HT]) between these cel...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2005-07-2903
更新日期:2006-02-01 00:00:00
abstract::Arsenic trioxide (As2O3) induces clinical remission in acute promyelocytic leukemia (APL) with minimal toxicity and apoptosis in APL-derived NB4 cells at low (1 to 2 micromol/L) concentration. We examined the basis for NB4 cell sensitivity to As2O3 to identify experimental conditions that would render other malignant ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1999-01-01 00:00:00
abstract::Essential thrombocythemia (ET) and polycythemia vera (PV) are characterized by persistent platelet activation. The mechanisms involved in their clearance are poorly characterized. In the present study, we report that leukocytes were actively involved in platelet disposal in 51 patients with ET and 30 with PV, but not ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-02-337337
更新日期:2011-09-22 00:00:00
abstract::Patients with severe aplastic anemia were conditioned with cyclophosphamide (200 mg/kg) and given marrow grafts from HLA-identical family members. Among 233 patients transplanted, 225 survived greater than or equal to 14 days and could be evaluated for engraftment. Forty-four of the 225 rejected their graft; 33 of the...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1986-12-01 00:00:00
abstract::Platelets stored as concentrates (PC) for 72 h at 22 degrees C develop a functional defect. Alterations in adenine nucleotides of platelets have been shown to affect platelet function. Adenine nucleotide content of platelets was measured before and after storage and a decrease of 27.1 /+- 1.7% (mean /+- SE) in ATP and...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1981-02-01 00:00:00
abstract::The etiopathologic basis of painful crisis in sickle cell anemia is largely unknown, and no objective criteria for its diagnosis and follow-up exist at present. We have studied 11 patients through 14 painful crises and observed a significant decrease of the densest fraction of red cells in 12 of the 14 crises as deter...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1984-08-01 00:00:00
abstract::Granulocyte colony-stimulating factor (G-CSF) is essential for the host response to bacterial infection by controlling neutrophil production in the bone marrow. The G-CSF receptor (G-CSFR) activates the Jak/STAT pathway, although little is understood about how these signals regulate basal and stress-induced granulopoi...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-02-003012
更新日期:2006-12-01 00:00:00
abstract::Histone deacetylase (HDAC) enzymatic activity has been linked to the transcription of DNA in cancers including multiple myeloma (MM). Therefore, HDAC inhibitors used alone and in combination are being actively studied as novel therapies in MM. In the present study, we investigated the preclinical activity of ACY-1215,...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-10-387365
更新日期:2012-03-15 00:00:00
abstract::Platelet-von Willebrand factor (VWF) interactions must be tightly regulated in order to promote effective hemostasis and prevent occlusive thrombus formation. However, it is unclear what role the inherent properties of the bond formed between the platelet receptor glycoprotein Ibα and the A1 domain of VWF play in thes...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2014-04-569392
更新日期:2014-12-11 00:00:00
abstract::Overactivated complement is a high-risk feature in hematopoietic stem cell transplant (HSCT) recipients with transplant-associated thrombotic microangiopathy (TA-TMA), and untreated patients have dismal outcomes. We present our experience with 64 pediatric HSCT recipients who had high-risk TA-TMA (hrTA-TMA) and multio...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2019004218
更新日期:2020-03-26 00:00:00