Update on clinical gene therapy for hemophilia.

abstract：:Peripheral blood samples from 57 children with newly diagnosed E-rosette-negative, surface-immunoglobulin negative acute lymphocytic leukemia (ALL) were studied for the presence of a leukemia-associated antigen (ALLA). Ficoll-Hypaque separated cells were tested using a rabbit antiserum to human null lymphoblasts and a...

journal_title：Blood

authors： Morgan E,Hsu CC

更新日期：1981-05-01 00:00:00

abstract：:Filamin A (FLNa), a dimeric actin cross-linking and scaffold protein with numerous intracellular binding partners, anchors the platelet adhesion glycoprotein (GP) Ib-IX-V receptor to actin cytoskeleton. We mapped the GPIbalpha binding site to a single domain of FLNa and resolved the structure of this domain and its in...

journal_title：Blood

authors： Nakamura F,Pudas R,Heikkinen O,Permi P,Kilpeläinen I,Munday AD,Hartwig JH,Stossel TP,Ylänne J

更新日期：2006-03-01 00:00:00

abstract：:Toll-like receptors orchestrate rapid local protective innate-immune responses to invading pathogens and optimize leukocyte priming of subsequent adaptive responses. Paradoxically, systemic excess of the TLR2 ligand, bacterial lipoprotein (BLP), suppresses peripheral inflammatory responses. Here, we demonstrate that t...

journal_title：Blood

authors： McKimmie CS,Moore M,Fraser AR,Jamieson T,Xu D,Burt C,Pitman NI,Nibbs RJ,McInnes IB,Liew FY,Graham GJ

更新日期：2009-04-30 00:00:00

abstract：:Approximately 20% of childhood B-precursor acute lymphoblastic leukemia (ALL) has a TEL-AML1 fusion gene, often in association with deletions of the nonrearranged TEL allele. TEL-AML1 gene fusion appears to be an initiating event and usually occurs before birth, in utero. This subgroup of ALL generally presents with l...

journal_title：Blood

authors： Ford AM,Fasching K,Panzer-Grümayer ER,Koenig M,Haas OA,Greaves MF

更新日期：2001-08-01 00:00:00

abstract：:Congenital thrombocytopenia may occur in isolation or accompanied by eczema and immunodeficiency, as part of the X-linked hereditary Wiskott-Aldrich syndrome (WAS). Because the clinical and immunologic picture of WAS is variable, particularly early in life, definite diagnosis cannot always be made in cases with a nega...

journal_title：Blood

authors： Puck JM,Siminovitch KA,Poncz M,Greenberg CR,Rottem M,Conley ME

更新日期：1990-06-15 00:00:00

abstract：:Patients with disseminated follicular non-Hodgkin's lymphoma (NHL) are only occasionally cured with standard chemotherapy regimens. Although most of these tumors are initially responsive to chemotherapy, in 40% to 70% of patients the lymphoma will eventually transform to an NHL of higher grade malignancy and a poorer ...

journal_title：Blood

authors： Schouten HC,Bierman PJ,Vaughan WP,Kessinger A,Vose JM,Weisenburger DD,Armitage JO

更新日期：1989-11-15 00:00:00

abstract：:Current dendritic cell (DC)-based vaccines are based on ex vivo-generated autologous DCs loaded with antigen prior to readministration into patients. A more direct and less laborious strategy is to target antigens to DCs in vivo via specific surface receptors. Therefore, we developed a humanized antibody, hD1V1G2/G4 (...

journal_title：Blood

authors： Tacken PJ,de Vries IJ,Gijzen K,Joosten B,Wu D,Rother RP,Faas SJ,Punt CJ,Torensma R,Adema GJ,Figdor CG

更新日期：2005-08-15 00:00:00

abstract：:Partially purified hemoglobin A2 has been examined for the existence of glycosylated components by isoelectric focusing and by acid agar gel electrophoresis. Bands analogous to the glycohemoglobin derivatives of hemoglobin A, hemoglobin-A1.a.b.c, were readily detected. Evidence that these minor bands are in fact glyco...

journal_title：Blood

authors： Tegos C,Beutler E

更新日期：1980-09-01 00:00:00

abstract：:Although the chromosomal localization (9q34) of the gene encoding the human form of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) and its exclusive expression in the liver have been established, the cells that produce this enzyme are yet to be determined. We investigated the ...

journal_title：Blood

authors： Uemura M,Tatsumi K,Matsumoto M,Fujimoto M,Matsuyama T,Ishikawa M,Iwamoto TA,Mori T,Wanaka A,Fukui H,Fujimura Y

更新日期：2005-08-01 00:00:00

abstract：:FLT3 is constitutively activated by internal tandem duplications (ITDs) in the juxtamembrane domain or by activation loop mutations in acute myeloid leukemia (AML). We tested the sensitivity of 8 activation loop mutations to the small molecule FLT3 inhibitor, MLN518. Each FLT3 activation loop mutant, including D835Y, ...

journal_title：Blood

authors： Clark JJ,Cools J,Curley DP,Yu JC,Lokker NA,Giese NA,Gilliland DG

更新日期：2004-11-01 00:00:00

abstract：:The Wiskott-Aldrich syndrome (WAS) is a severe immunodeficiency and platelet deficiency disease arising from mutation(s) in the WASP gene, which in normal cells encodes an intracellular protein able to interact with other proteins relevant to the control of cytoskeleton organization. Immunodeficiency is mainly due to ...

journal_title：Blood

authors： Gallego MD,Santamaría M,Peña J,Molina IJ

更新日期：1997-10-15 00:00:00

abstract：:Homeostatic mechanism by which peripheral T-cell subsets are maintained in vivo remains largely unknown. Using a T-cell proliferation model under lymphopenic settings, we now demonstrate that gammadelta T cells limit CD8 T-cell expansion but not the initial proliferation after transfer into lymphopenic recipients. Int...

journal_title：Blood

authors： Do JS,Min B

更新日期：2009-06-18 00:00:00

abstract：:Macrophages are motile leukocytes, targeted by HIV-1, thought to play a critical role in host dissemination of the virus. However, whether infection impacts their migration capacity remains unknown. We show that 2-dimensional migration and the 3-dimensional (3D) amoeboid migration mode of HIV-1-infected human monocyte...

journal_title：Blood

authors： Vérollet C,Souriant S,Bonnaud E,Jolicoeur P,Raynaud-Messina B,Kinnaer C,Fourquaux I,Imle A,Benichou S,Fackler OT,Poincloux R,Maridonneau-Parini I

更新日期：2015-03-05 00:00:00

abstract：:The anti-CD20 monoclonal antibody (mAb) rituximab has revolutionized the treatment of B-cell malignancies. This unprecedented success has not only substantially changed the mindset of the clinical community about the ability of mAb to improve outcomes but has catalyzed the interest in the pharmaceutical industry to de...

journal_title：Blood

authors： Alduaij W,Illidge TM

更新日期：2011-03-17 00:00:00

abstract：:We investigated the clinical activity of the farnesyl transferase inhibitor R115777 in 22 patients with chronic myelogenous leukemia (CML) in chronic, accelerated, or blastic phase and in 8 patients with myelofibrosis (MF) and 10 patients with multiple myeloma (MM). R115777 was administered at 600 mg orally twice dail...

journal_title：Blood

authors： Cortes J,Albitar M,Thomas D,Giles F,Kurzrock R,Thibault A,Rackoff W,Koller C,O'Brien S,Garcia-Manero G,Talpaz M,Kantarjian H

更新日期：2003-03-01 00:00:00

abstract：:Pregnancy can precipitate thrombotic thrombocytopenic purpura (TTP). We present a prospective study of TTP cases from the United Kingdom Thrombotic Thrombocytopenic Purpura (UK TTP) Registry with clinical and laboratory data from the largest cohort of pregnancy-associated TTP and describe management through pregnancy,...

journal_title：Blood

authors： Scully M,Thomas M,Underwood M,Watson H,Langley K,Camilleri RS,Clark A,Creagh D,Rayment R,Mcdonald V,Roy A,Evans G,McGuckin S,Ni Ainle F,Maclean R,Lester W,Nash M,Scott R,O Brien P,collaborators of the UK TTP Registr

更新日期：2014-07-10 00:00:00

abstract：:Recently, the interleukin-2 receptor (IL-2R) was shown to be present on human neutrophils, and IL-2-neutrophil interactions are believed to be important in both tumor rejection and increased susceptibility to bacterial infections. Furthermore, neutrophils have been shown to synthesize host defense proteins, such as cy...

journal_title：Blood

authors： Girard D,Gosselin J,Heitz D,Paquin R,Beaulieu AD

更新日期：1995-08-01 00:00:00

abstract：:The use of recombinant adeno-associated virus (rAAV) vectors provides a new strategy to investigate the role of specific regulatory elements and trans-acting factors in globin gene expression. We linked hypersensitivity site 2 (HS2) from the locus control region (LCR) to a A gamma-globin gene (A gamma*) mutationally m...

journal_title：Blood

authors： Miller JL,Walsh CE,Ney PA,Samulski RJ,Nienhuis AW

更新日期：1993-09-15 00:00:00

abstract：:Surprisingly little is known about the interaction of human blood mononuclear cells with viruses. Here, we show that monocytes are the predominant cell type infected when peripheral blood mononuclear cells are exposed to viruses ex vivo. Remarkably, infection with vesicular stomatitis virus, vaccinia virus, and a vari...

journal_title：Blood

authors： Hou W,Gibbs JS,Lu X,Brooke CB,Roy D,Modlin RL,Bennink JR,Yewdell JW

更新日期：2012-03-29 00:00:00

abstract：:Immunizing bone marrow donors prior to bone marrow transplant (BMT) has the potential for adoptively transferring specific immunity against opportunistic pathogens. Studies have shown that long-term antibody production occurs in the bone marrow and that specific humoral immunity may be transferred from donor to recipi...

journal_title：Blood

authors： Slifka MK,Whitmire JK,Ahmed R

更新日期：1997-09-01 00:00:00

abstract：:The Gardos channel is a Ca(2+)-sensitive, intermediate conductance, potassium selective channel expressed in several tissues including erythrocytes and pancreas. In normal erythrocytes, it is involved in cell volume modification. Here, we report the identification of a dominantly inherited mutation in the Gardos chann...

journal_title：Blood

authors： Rapetti-Mauss R,Lacoste C,Picard V,Guitton C,Lombard E,Loosveld M,Nivaggioni V,Dasilva N,Salgado D,Desvignes JP,Béroud C,Viout P,Bernard M,Soriani O,Vinti H,Lacroze V,Feneant-Thibault M,Thuret I,Guizouarn H,Badens C

更新日期：2015-09-10 00:00:00

abstract：:NF-IL6 was originally identified as a DNA binding protein regulating interleukin-1 (IL-1)-stimulated IL-6 expression. Direct cloning of NF-IL6 showed its homology with C/EBP, a hepatocyte- and adipocyte-specific transcription factor. This study showed that the expression of NF-IL6 messenger RNA (mRNA) increased marked...

journal_title：Blood

authors： Natsuka S,Akira S,Nishio Y,Hashimoto S,Sugita T,Isshiki H,Kishimoto T

更新日期：1992-01-15 00:00:00

abstract：:Bernard-Soulier syndrome (BSS) platelets, which lack the membrane glycoprotein complex Ib-IX, do not adhere to subendothelium. The adhesion of platelets from two patients with BSS to subendothelial microfibrils (MFs) and type I collagen was compared in an in vitro assay adapted to patients with low platelet count. Wit...

journal_title：Blood

authors： Fauvel-Lafève F,Tabaka V,Caen JP,Legrand YJ

更新日期：1993-10-01 00:00:00

abstract：:The human peripheral B-cell compartment displays a large population of immunoglobulin M-positive, immunoglobulin D-positive CD27(+) (IgM(+)IgD(+)CD27(+)) "memory" B cells carrying a mutated immunoglobulin receptor. By means of phenotypic analysis, complementarity-determining region 3 (CDR3) spectratyping during a T-in...

journal_title：Blood

authors： Weller S,Braun MC,Tan BK,Rosenwald A,Cordier C,Conley ME,Plebani A,Kumararatne DS,Bonnet D,Tournilhac O,Tchernia G,Steiniger B,Staudt LM,Casanova JL,Reynaud CA,Weill JC

更新日期：2004-12-01 00:00:00

abstract：:The expression of a number of blood coagulation factors (F) (FX, FIX, FVIII, FVII, alpha-, beta-, gamma-fibrinogen chains, protein C, and antithrombin III [AT III]) was analyzed at RNA and protein level in 5- to 10-week-old human embryos and fetuses. FX, FIX, and FVII were also analyzed at protein level. Total and pol...

journal_title：Blood

authors： Hassan HJ,Leonardi A,Chelucci C,Mattia G,Macioce G,Guerriero R,Russo G,Mannucci PM,Peschle C

更新日期：1990-09-15 00:00:00

abstract：:The glycoprotein CD47 (integrin-associated protein, IAP) is present on the surface of virtually all cells, including red blood cells (RBCs). CD47 acts like a marker of self by ligating the macrophage inhibitory receptor signal regulatory protein alpha (SIRPalpha). In this manner mild reactivity of wild-type RBCs with ...

journal_title：Blood

authors： Oldenborg PA,Gresham HD,Chen Y,Izui S,Lindberg FP

更新日期：2002-05-15 00:00:00

abstract：:Lymphomas were studied in kindreds with autoimmune lymphoproliferative syndrome (ALPS; Canale-Smith syndrome), a disorder of lymphocyte homeostasis usually associated with germline Fas mutations. Fas (CD95/APO-1) is a cell surface receptor that initiates programmed cell death, or apoptosis, of activated lymphocytes. L...

journal_title：Blood

authors： Straus SE,Jaffe ES,Puck JM,Dale JK,Elkon KB,Rösen-Wolff A,Peters AM,Sneller MC,Hallahan CW,Wang J,Fischer RE,Jackson CE,Lin AY,Bäumler C,Siegert E,Marx A,Vaishnaw AK,Grodzicky T,Fleisher TA,Lenardo MJ

更新日期：2001-07-01 00:00:00

abstract：:Prediagnostic serum concentration of soluble CD30 (sCD30), a marker for chronic B-cell stimulation, has been associated with increased risk of developing AIDS-related non-Hodgkin lymphoma (NHL) in a recent study of HIV(+) patients. To investigate among healthy persons whether serum sCD30 is associated with NHL risk, w...

journal_title：Blood

authors： Purdue MP,Lan Q,Martinez-Maza O,Oken MM,Hocking W,Huang WY,Baris D,Conde B,Rothman N

更新日期：2009-09-24 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：:The regulation of plasmin generation on cell surfaces is of critical importance in the control of vascular homeostasis. Cell-derived microparticles participate in the dissemination of biological activities. However, their capacity to promote plasmin generation has not been documented. In this study, we show that endot...

journal_title：Blood

authors： Lacroix R,Sabatier F,Mialhe A,Basire A,Pannell R,Borghi H,Robert S,Lamy E,Plawinski L,Camoin-Jau L,Gurewich V,Angles-Cano E,Dignat-George F

更新日期：2007-10-01 00:00:00