Development of virus vectors for gene therapy of beta chain hemoglobinopathies: flanking with a chromatin insulator reduces gamma-globin gene silencing in vivo.

abstract：:Thrombin Metz and normal thrombin, resulting from activation of the respective prothrombins by factor Xa in the presence of calcium, phospholipid, and factor Va, were purified by chromatography on sulfopropyl Sephadex. By physicochemical criteria, thrombin Metz is identical to normal thrombin. Its functional propertie...

journal_title：Blood

authors： Rabiet MJ,Jandrot-Perrus M,Boissel JP,Elion J,Josso F

更新日期：1984-04-01 00:00:00

abstract：:An inhibitor of procoagulant and fibrinolytic enzymes was derived from cabbage seeds by a procedure using acetone precipitation, ion-exchange chromatography, and gel filtration. The cabbage seed inhibitor was a 10-Kd monomeric protein with intrachain disulfide bonds. This preparation prevented clot formation in whole ...

journal_title：Blood

authors： Carter TH,Everson BA,Ratnoff OD

更新日期：1990-01-01 00:00:00

abstract：:Members of the 14-3-3 family of proteins function as adapters/modulators that recognize phosphoserine/phosphothreonine-based binding motifs in many intracellular proteins and play fundamental roles in signal transduction pathways of eukaryotic cells. In platelets, 14-3-3 plays a wide range of regulatory roles in phosp...

journal_title：Blood

authors： Chen Y,Ruggeri ZM,Du X

更新日期：2018-05-31 00:00:00

abstract：:Autosomal recessive STAT1 deficiency is associated with impaired cellular responses to interferons and susceptibility to intracellular bacterial and viral infections. We report here a new form of partial STAT1 deficiency in 2 siblings presenting mycobacterial and viral diseases. Both carried a homozygous missense muta...

journal_title：Blood

authors： Kong XF,Ciancanelli M,Al-Hajjar S,Alsina L,Zumwalt T,Bustamante J,Feinberg J,Audry M,Prando C,Bryant V,Kreins A,Bogunovic D,Halwani R,Zhang XX,Abel L,Chaussabel D,Al-Muhsen S,Casanova JL,Boisson-Dupuis S

更新日期：2010-12-23 00:00:00

abstract：:Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative disorders characterized by an increased incidence of thrombo-hemorrhagic complications. The acquired somatic Janus kinase 2 (JAK2) V617F mutation is present in the majority of PV and ET patients. Because aberrant protein Tyr-phosp...

journal_title：Blood

authors： Randi ML,Brunati AM,Scapin M,Frasson M,Deana R,Magrin E,Fabris F,Donella-Deana A

更新日期：2010-01-21 00:00:00

abstract：:Approximately 40% of children with acute myeloid leukemia (AML) who respond to initial therapy subsequently relapse. Multidimensional flow cytometry employing a standardized panel of monoclonal antibodies enables the detection of small numbers of occult leukemic cells that persist during therapy using technology adapt...

journal_title：Blood

authors： Sievers EL,Lange BJ,Alonzo TA,Gerbing RB,Bernstein ID,Smith FO,Arceci RJ,Woods WG,Loken MR

更新日期：2003-05-01 00:00:00

abstract：:Peripheral blood leukemia cells from patients with acute monoblastic leukemia (AMoL) were tested for killer cell activity against target cells that detected natural killer cell-mediated or monocyte-mediated spontaneous cytotoxicity. The fibrosarcoma cell line Wehi 164, pretreated with actinomycin D to induce susceptib...

journal_title：Blood

authors： Ziegler-Heitbrock HW,Munker R,Thiel E,Krebs I,Riethmüller G

更新日期：1985-01-01 00:00:00

abstract：:Production of a red blood cell's hemoglobin depends on mitochondrial heme synthesis. However, mature red blood cells are devoid of mitochondria and rely on glycolysis for ATP production. The molecular basis for the selective elimination of mitochondria from mature red blood cells remains controversial. Recent evidence...

journal_title：Blood

authors： Kundu M,Lindsten T,Yang CY,Wu J,Zhao F,Zhang J,Selak MA,Ney PA,Thompson CB

更新日期：2008-08-15 00:00:00

abstract：:Factor VIII (FVIII) functions as a cofactor within the intrinsic pathway of blood coagulation. Quantitative or qualitative deficiencies of FVIII result in the inherited bleeding disorder hemophilia A. Expression of FVIII (domain structure A1-A2-B-A3-C1-C2) in heterologous mammalian systems is 2 to 3 orders of magnitud...

journal_title：Blood

authors： Miao HZ,Sirachainan N,Palmer L,Kucab P,Cunningham MA,Kaufman RJ,Pipe SW

更新日期：2004-05-01 00:00:00

abstract：:Self-renewal is a feature of cancer and can be assessed by cell transplantation into immune-compromised or immune-matched animals. However, studies in zebrafish have been severely limited by lack of these reagents. Here, Myc-induced T-cell acute lymphoblastic leukemias (T-ALLs) have been made in syngeneic, clonal zebr...

journal_title：Blood

authors： Smith AC,Raimondi AR,Salthouse CD,Ignatius MS,Blackburn JS,Mizgirev IV,Storer NY,de Jong JL,Chen AT,Zhou Y,Revskoy S,Zon LI,Langenau DM

更新日期：2010-04-22 00:00:00

abstract：:The reported outcome of hematopoietic stem cell transplantation (HSCT) from HLA-mismatched family members has been inconsistent. The object of this study was to evaluate the true impact of HLA-mismatch by using recent data from a homogenous population, excluding HSCT procedures that used graft manipulations, and by co...

journal_title：Blood

authors： Kanda Y,Chiba S,Hirai H,Sakamaki H,Iseki T,Kodera Y,Karasuno T,Okamoto S,Hirabayashi N,Iwato K,Maruta A,Fujimori Y,Furukawa T,Mineishi S,Matsuo K,Hamajima N,Imamura M

更新日期：2003-08-15 00:00:00

abstract：:We show the molecular and functional characterization of a novel population of lineage-negative CD34-negative (Lin(-)CD34(-)) hematopoietic stem cells from chronic myelogenous leukemia (CML) patients at diagnosis. Molecular karyotyping and quantitative analysis of BCR-ABL transcript demonstrated that approximately one...

journal_title：Blood

authors： Lemoli RM,Salvestrini V,Bianchi E,Bertolini F,Fogli M,Amabile M,Tafuri A,Salati S,Zini R,Testoni N,Rabascio C,Rossi L,Martin-Padura I,Castagnetti F,Marighetti P,Martinelli G,Baccarani M,Ferrari S,Manfredini R

更新日期：2009-12-10 00:00:00

abstract：:In this study, we show that microRNA-150 (miR-150) is significantly downregulated in advanced cutaneous T-cell lymphoma (CTCL), and that this downregulation is strongly associated with tumor invasion/metastasis. Inoculation of CTCL cell lines into nonobese diabetic/Shi-scid interleukin 2γ (IL-2γ) null mice led to CTCL...

journal_title：Blood

authors： Ito M,Teshima K,Ikeda S,Kitadate A,Watanabe A,Nara M,Yamashita J,Ohshima K,Sawada K,Tagawa H

更新日期：2014-03-06 00:00:00

abstract：:Typical acute promyelocytic leukemia (APL) is associated with expression of the PML-RARalpha fusion protein and responsiveness to treatment with all-trans retinoic acid (ATRA). A rare, but recurrent, APL has been described that does not respond to ATRA treatment and is associated with a variant chromosomal translocati...

journal_title：Blood

authors： Guidez F,Ivins S,Zhu J,Söderström M,Waxman S,Zelent A

更新日期：1998-04-15 00:00:00

abstract：:Deregulation of Notch signaling has been linked to the development of T-cell leukemias and several solid malignancies. Yet, it is unknown whether Notch signaling is involved in the pathogenesis of mycosis fungoides and Sézary syndrome, the most common subtypes of cutaneous T-cell lymphoma. By immunohistochemistry of 4...

journal_title：Blood

authors： Kamstrup MR,Gjerdrum LM,Biskup E,Lauenborg BT,Ralfkiaer E,Woetmann A,Ødum N,Gniadecki R

更新日期：2010-10-07 00:00:00

abstract：:The malignant monoclonal population in hairy cell leukemia (HCL) has been variously ascribed to be of myeloid, B, or even T cell origin. Recent data have been interpreted as suggesting that hairy cells (HC) may concomitantly or serially express both B and T surface determinants, a phenomenon which, if verified, would ...

journal_title：Blood

authors： Han T,Dadey B,Pollard C,Barcos M,Bloom ML,Minowada J,Ozer H

更新日期：1984-04-01 00:00:00

abstract：:As a mediator of neurogenic inflammation and pain, we hypothesized that levels of the neuropeptide Substance P (SP) would be elevated in patients with sickle cell disease (SCD) with vaso-occlusive pain crisis. SP is a known stimulator of tumor necrosis factor-alpha (TNF-alpha) release and a promoter of interleukin-8 (...

journal_title：Blood

authors： Michaels LA,Ohene-Frempong K,Zhao H,Douglas SD

更新日期：1998-11-01 00:00:00

abstract：:Regulatory molecules produced by stromal cells are often membrane bound until cleaved by matrix metalloproteinases (MMPs); cleavage can either activate or inactivate regulatory functions. We report here that marrow stromal cells induce the expression of MMP-9 in monocytes. Induction was contact independent and could b...

journal_title：Blood

authors： Iwata M,Pillai M,Ramakrishnan A,Hackman RC,Deeg HJ,Opdenakker G,Torok-Storb B

更新日期：2007-01-01 00:00:00

abstract：:Two sisters in whom a diagnosis of Fanconi's anemia was made at ages 12 and 18 subsequently developed acute nonlymphocytic leukemia (ANLL). A third sibling had previously died at age 11 of apparent sepsis. Both sisters had cytogenetic studies that showed increased chromosomal breakage and a 46,XX karyotype, but subseq...

journal_title：Blood

authors： Stivrins TJ,Davis RB,Sanger W,Fritz J,Purtilo DT

更新日期：1984-07-01 00:00:00

abstract：:There is growing evidence that lymphocyte trafficking contributes to the clinical course of chronic lymphocytic leukemia (CLL), but to date, only static in vitro cultures have been used to study these phenomena. To address this lack of data, we have developed a dynamic in vitro model in which CLL cells experience shea...

journal_title：Blood

authors： Walsby E,Buggins A,Devereux S,Jones C,Pratt G,Brennan P,Fegan C,Pepper C

更新日期：2014-06-05 00:00:00

abstract：:Leukocyte rolling precedes firm adhesion and emigration in inflammatory cell recruitment. Both P-selectin, an endothelial lectin that binds to sialylated O-glycans containing sialyl-Lewisx (sLex) on the granulocyte surface, and leukocyte L-selectin have been shown to mediate leukocyte rolling in vivo. Here, we investi...

journal_title：Blood

authors： Ley K,Zakrzewicz A,Hanski C,Stoolman LM,Kansas GS

更新日期：1995-06-15 00:00:00

abstract：:Recent phenotypic analysis of plasma cells showed that normal plasma cells do express the B-cell lineage-specific molecule CD19, but their malignant counterpart (myeloma cells) are CD19-. To clarify the meaning of loss of CD19 antigen on myeloma cells, we first compared the expression of CD19 and Pax-5 genes among B c...

journal_title：Blood

authors： Mahmoud MS,Huang N,Nobuyoshi M,Lisukov IA,Tanaka H,Kawano MM

更新日期：1996-05-15 00:00:00

abstract：:We demonstrate a methodology for tracing the clonal history of hematopoietic stem and progenitor cells (HSPCs) behavior in live tissues in 4 dimensions (4D). This integrates genetic combinatorial marking using lentiviral vectors encoding various fluorescent proteins (FPs) with advanced imaging methods. Five FPs: Cerul...

journal_title：Blood

authors： Malide D,Métais JY,Dunbar CE

更新日期：2012-12-20 00:00:00

abstract：:Thalassemia is a congenital hemolytic disorder caused by a partial or complete deficiency of alpha- or beta-globin chain synthesis. Homozygous carriers of beta-globin gene defects suffer from severe anemia and other serious complications from early childhood. The disease is treated by chronic blood transfusion. Howeve...

journal_title：Blood

authors： Eldor A,Rachmilewitz EA

更新日期：2002-01-01 00:00:00

abstract：:The administration of greater than or equal to 5 micrograms interleukin-7 (IL-7) twice a day to mice for 4 to 7 days increased by twofold to fivefold the total number of splenic and peripheral blood leukocytes, but did not appreciably increase bone marrow (BM) cellularity. This regimen of IL-7 administration also resu...

journal_title：Blood

authors： Damia G,Komschlies KL,Faltynek CR,Ruscetti FW,Wiltrout RH

更新日期：1992-03-01 00:00:00

abstract：:The primary objective was to describe the prevalence and characteristics of microbiologically defined infections and infection-related mortality (IRM) in 492 children with acute myeloid leukemia enrolled on CCG 2961. Secondary objectives were to determine the relationship between demographic, disease-related, and ther...

journal_title：Blood

authors： Sung L,Lange BJ,Gerbing RB,Alonzo TA,Feusner J

更新日期：2007-11-15 00:00:00

abstract：:The hereditary stomatocytoses are a series of dominantly inherited hemolytic anemias in which the permeability of the erythrocyte membrane to monovalent cations is pathologically increased. The causative mutations for some forms of hereditary stomatocytosis have been found in the transporter protein genes, RHAG and SL...

journal_title：Blood

authors： Flatt JF,Guizouarn H,Burton NM,Borgese F,Tomlinson RJ,Forsyth RJ,Baldwin SA,Levinson BE,Quittet P,Aguilar-Martinez P,Delaunay J,Stewart GW,Bruce LJ

更新日期：2011-11-10 00:00:00

abstract：:The PI3K/Akt/mTOR pathway has emerged as a critical regulator of dendritic cell (DC) development and function. The kinase mTOR is found in 2 distinct complexes, mTORC1 and mTORC2. In this study, we show that mTORC1 but not mTORC2 is required for epidermal Langerhans cell (LC) homeostasis. Although the initial seeding ...

journal_title：Blood

authors： Kellersch B,Brocker T

更新日期：2013-01-10 00:00:00

abstract：:A substantial body of published data suggests activation of lineage-specific genes in multipotential hemopoietic cells before their unilineage commitment. Because the behavior and plasticity of cells isolated in vitro away from microenvironmental constraints exercised in vivo may be altered, one wonders whether simila...

journal_title：Blood

authors： Papayannopoulou T,Priestley GV,Rohde A,Peterson KR,Nakamoto B

更新日期：2000-02-15 00:00:00

abstract：:Administration of hematopoietic growth factors is being used increasingly to obtain populations of blood progenitor/stem cells (PBPC) for clinical transplantation. Here we examined the effect of combining stem cell factor (SCF ) and granulocyte colony-stimulating factor (G-CSF ) versus G-CSF alone in a randomized clin...

journal_title：Blood

authors： Begley CG,Basser R,Mansfield R,Thomson B,Parker WR,Layton J,To B,Cebon J,Sheridan WP,Fox RM,Green MD

更新日期：1997-11-01 00:00:00