Abstract：
:The granulocyte defects of Chediak-Higashi syndrome (CHS) include neutropenia, characteristic giant lysosomal granule morphology, and functionally abnormal cell motility, degranulation, and bacterial killing. Findings of elevated levels of adenosine 3', 5' cyclic monophosphate nucleotide (cAMP) and of concanavalin A (Con A) capping have suggested a pathogenic role of a cyclic-nucleotide-related defect in microtubule polymerization, but not all patients exhibit these abnormalities. In order to test which defects derive from the cells' genetic program and which from the host environment, we examined granulocytes produced by CHS bone marrow progenitors in long-term in vitro bone marrow cultures. These cells exhibited the characteristic giant-granule morphology and defective cell motility of CHS. However, culture-derived CHS granulocytes had normal cAMP contents and normal spontaneous capping of Con A. Granulopoiesis diminished dramatically after five weeks in culture, with accompanying autophagocytosis by mononuclear phagocytes. In mixing experiments, the phenotype of the mature granulocytes corresponded to the genotype of the hematopoietic component of the culture rather than the stroma. These results indicate that the hallmark giant-granule morphology and cell motility defect of CHS are expressions of the genetic program of the hematopoietic cells. However the abnormalities in resting cyclic nucleotide levels and in Con-A capping may be secondary manifestations of the disease and are not essential to the pathogenesis of the chemotactic defect.
journal_name
Exp Hematoljournal_title
Experimental hematologyauthors
Newburger PE,Speier C,Stock JL,Perrine SP,Greenberger JSsubject
Has Abstractpub_date
1985-02-01 00:00:00pages
117-22issue
2eissn
0301-472Xissn
1873-2399journal_volume
13pub_type
杂志文章abstract：:Serum erythropoietin (Epo) levels are depressed in anemic AIDS patients relative to controls. The basis for abnormal regulation of Epo has not been defined. The hepatoma cell line Hep3B produces Epo in response to hypoxia and serves as a model for the study of Epo regulation. Hep3B cells are infectible with human immu...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1993-05-01 00:00:00
abstract：:This review of adjunctive therapy with subcutaneous granulocyte-colony stimulating factor (G-CSF) to patients with acute myocardial infarction (AMI) focus on the cardioprotective effects and potential mechanisms of G-CSF and discuss the therapeutic potential of G-CSF. All clinical trials published in peer-reviewed jou...
journal_title：Experimental hematology
pub_type： 杂志文章,评审
doi：10.1016/j.exphem.2008.01.010
更新日期：2008-06-01 00:00:00
abstract：:Circulating CD14(+) monocytes are originated from hematopoietic stem cells in the bone marrow and believed to be committed precursors for phagocytes, such as macrophages. Recently, we have reported a primitive cell population termed monocyte-derived multipotential cells (MOMCs), which has a fibroblast-like morphology ...
journal_title：Experimental hematology
pub_type： 杂志文章,评审
doi：10.1016/j.exphem.2010.03.015
更新日期：2010-07-01 00:00:00
abstract：OBJECTIVE:Generation and expansion of pluripotent unrestricted somatic stem cells (USSCs) and mesenchymal cells (MSCs) from human cord blood as well their functional characterization were assessed. METHODS:USSC generation was initiated from fresh and cryopreserved cord blood (CB). Culture conditions and functional ass...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2006.07.011
更新日期：2006-11-01 00:00:00
abstract：:Adherent cells from mouse bone marrow have been shown to promote the in vitro growth of the AVRij-1 tumour cell line. The experiments presented here suggest that the adherent cells involved in this phenomenon are the progeny of bone marrow derived fibroblastoid colony forming units. The latter were characterized by me...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1979-04-01 00:00:00
abstract：:Ouabain has been shown to increase the number of clonally derived erythroid stem cells, CFUE and BFUE, from normal murine bone marrow. We report here that digoxin and theophylline also enhance erythroid stem cell colony formation, in the presence of suboptimal concentrations of erythropoietin (Ep) (0.01 IU/ml) in meth...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1982-09-01 00:00:00
abstract：:Arsenic trioxide (As2O3) has been demonstrated to be effective for the treatment of acute promyelocytic leukemia (APL) and to inhibit proliferation and produce apoptosis in the APL cell line NB4. To determine if As2O3 might be useful for the treatment of other lineages, we investigated the effects of As2O3 on viabilit...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/s0301-472x(99)00014-4
更新日期：1999-05-01 00:00:00
abstract：:The anemia of Belgrade b/b rats has been shown to be due to intracellular iron deficiency. The aim of this study of erythropoiesis at the progenitor cell level in these rats was to determine if a defect is present in the early phase of red cell production. Both erythroid colony-forming unit (CFU-E)- and erythroid burs...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1989-08-01 00:00:00
abstract：OBJECTIVE:Murine marrow cells are capable of repopulating skeletal muscle fibers. A point of concern has been the "robustness" of such conversions. We have investigated the impact of type of cell delivery, muscle injury, nature of delivered cell, and stem cell mobilizations on marrow-to-muscle conversion. METHODS:We t...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2004.02.007
更新日期：2004-05-01 00:00:00
abstract：OBJECTIVE:Recent clinical trials of adoptive immunotherapy showed diminished reactivity of human T cells upon ex vivo manipulation. For a safe and effective clinical application of human T cells, it is necessary to improve ex vivo manipulation procedures and evaluate their impact on in vivo functionality. However, ther...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2006.09.018
更新日期：2007-01-01 00:00:00
abstract：:To characterize hematopoietic cell biology, many investigators have used protocols that enrich for primitive hematopoietic stem cells (PHSC). In this study, we quantified the long-term repopulating ability (LTRA) of enriched and discarded fractions of PHSC from day-14 murine fetal liver using the competitive repopulat...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1995-08-01 00:00:00
abstract：OBJECTIVE:Mechanisms of T-cell stimulation by Flt3 ligand (Flt3L) and granulocyte-macrophage colony-stimulating factor (GM-CSF) remain unclear. Herein, we compared the effects of Flt3L and GM-CSF on the expansion of dendritic cells (DC) and T-cell subsets and cytokine expression. METHODS:Naïve and effector/memory T ce...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/s0301-472x(01)00722-6
更新日期：2001-10-01 00:00:00
abstract：OBJECTIVE:Recent reports have shown that killer immunoglobulin-like receptors (KIR) and KIR ligand (KIR-L) genotype play a role in the pathophysiology of autoimmune disorders. Certain KIR/KIR-L combinations might convey a predisposition to immune-mediated bone marrow failure. Examining the genotypic makeup of human leu...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2005.07.005
更新日期：2005-11-01 00:00:00
abstract：:Rats with diets containing 0.2% propylthiouracil (PTU) throughout gestation had progeny with persistent cyanosis and high neonatal mortality. Histological and histochemical studies failed to reveal lung abnormalities in these pups. Studies of the blood of PTU-fed dams demonstrated that hemoglobin, packed cell volume, ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1982-01-01 00:00:00
abstract：OBJECTIVE:The tumor suppressor p15Ink4b (Ink4b) is a cell-cycle inhibitor that is inactivated in a high percentage of acute myeloid leukemia and myeloid dysplasia syndrome cases. Despite this, the role of Ink4b in hematopoiesis remains unclear. Here we examined the role of Ink4b in blood cell formation using Ink4b-defi...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2006.11.005
更新日期：2007-03-01 00:00:00
abstract：OBJECTIVES:To analyze the consequences of lymphocyte donations on lymphopoiesis in donors having previously undergone hematopoietic stem cell collection for allogeneic stem cell transplantation. METHODS:Repeated analysis of leukocyte subsets in the peripheral blood of 76 hematopoietic stem cell donors undergoing lymph...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2004.07.021
更新日期：2004-11-01 00:00:00
abstract：:The present studies evaluated effects of recombinant human (rhu) and murine (rmu) flt3 ligand (flt3-L) on colony formation by subsets of myeloid stem and progenitor cells present in low-density (LD) and cell-sorted CD34 hu cord blood (CB) and bone marrow (BM) cells and unseparated mu BM cells. By itself, flt3-L had we...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1995-09-01 00:00:00
abstract：OBJECTIVE:The purpose of this study was to evaluate the repopulating properties of bone marrow (BM) from mice irradiated during embryonic and adult stages of development. MATERIALS AND METHODS:Four-day-old embryos, 17-day-old fetuses, and 12-week-old mice were irradiated with of 1 or 3 Gy of x-rays. At 3 and 9 months ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/s0301-472x(99)00128-9
更新日期：2000-01-01 00:00:00
abstract：:Between 30 and 40% of the CSF present in agar was depleted after culture for 1 week with 50,000 nucleated murine bone marrow cells. No statistically significant difference could be detected between this depletion and the extent of depletion of CSF when the amount present was halved. There was no evidence that toxic or...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1977-03-01 00:00:00
abstract：:Data in this report determined the effect of a single injection of recombinant interleukin 1 alpha (rIL-1) prior to irradiation of B6D2F1 mice on the recovery of colony-forming cells (CFC) at early and late times after sublethal and lethal doses of radiation. Injection of rIL-1 promoted an earlier recovery of mature c...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1988-10-01 00:00:00
abstract：:We examined the effect of vesnarinone, an oral cardiotonic, on the growth and differentiation of human myeloid leukemia cells. Vesnarinone alone markedly induced erythroid differentiation of HEL cells. All-trans-retinoic acid also induced erythroid differentiation of the cells, and the differentiation was greatly enha...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1996-01-01 00:00:00
abstract：:An increase in triglyceride synthesis has been observed in cultures of human bone marrow fibroblasts after the cells reach confluence. The addition of hydrocortisone further enhances triglyceride synthesis. Conditioned medium from confluent cultures also stimulates adipogenesis, probably mediated by a factor released ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1987-03-01 00:00:00
abstract：OBJECTIVE:Progenipoietin-1 is an agonist of both the granulocyte colony-stimulating factor and fetal liver tyrosine kinase-3 receptors capable of inducing the proliferation of multiple hematopoietic cell lineages. The potential of progenipoietin-1 to mobilize transplantable hematopoietic stem cells into the peripheral ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/s0301-472x(01)00675-0
更新日期：2001-08-01 00:00:00
abstract：:Mast cell (MC)-fibroblast-immunocompetent cell interactions may play a role in the inflammatory and fibrotic processes present in chronic graft-vs.-host disease (cGVHD). Interactions between these cell types were examined in both murine and human cGVHD models. To this purpose, cell supernatants from mice or humans wit...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1997-03-01 00:00:00
abstract：OBJECTIVE:For approximately 5% of autologous transplant recipients and a higher proportion of allogeneic transplant recipients, low level and delayed platelet engraftment is an ongoing problem. Mesenchymal stem cells (MSC), which can be derived from bone marrow as well as other organs, are capable of differentiation in...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/s0301-472x(03)00042-0
更新日期：2003-05-01 00:00:00
abstract：:Recently, several human bone marrow stromal cell lines have established and produced hematopoietic growth factors. One of these factors, a burst-promoting activity (BPA), was purified from 6 liters of serum-free conditioned medium cultured from stromal cell line KM-102, which was stimulated by phorbol myristate acetat...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1988-08-01 00:00:00
abstract：:The in vitro and in vivo effects of murine recombinant interferon-gamma (rIFN-gamma) on hematopoietic and immune parameters of normal mice and of mice bearing metastatic variant Lewis lung carcinoma (LLC-C3) tumors were assessed. The in vitro addition of rIFN-gamma to bone marrow or spleen cells from normal and LLC-C3...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1988-05-01 00:00:00
abstract：:Sustained NF-κB activation is often observed in acute myeloid leukemia (AML); therefore, proteasome inhibition has been proposed to efficiently target AML cells. In this study, we questioned whether leukemic stem cell-enriched CD34(+) cells are sensitive to the proteasome inhibitor bortezomib. Surprisingly, we observe...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2013.02.002
更新日期：2013-06-01 00:00:00
abstract：OBJECTIVE:To investigate the role of perforin-mediated cell apoptosis in murine models of immune-mediated bone marrow (BM) failure. MATERIALS AND METHODS:We compared C57BL/6J (B6) mice carrying a perforin gene deletion (Prf(-/-)) with wild-type (WT) controls for cellular composition in lymphohematopoietic tissues. Lym...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2008.12.001
更新日期：2009-04-01 00:00:00
abstract：:Colony forming unit (CFU) assays were developed for feline granulocyte-macrophage (CFUGM), early erythroid (day 2 CFUE), and late erythroid (day 7 CFUE) colonies in methylcellulose medium. Feline CFUGM and both day 2 and day 7 CFUE were enhanced by feline macrophage conditioned medium and late CFUE often were intimate...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1981-11-01 00:00:00
