The role of the D1 domain of the von Willebrand factor propeptide in multimerization of VWF.

abstract：:We report here 7 new mutations in the ADAMTS13 gene responsible for Upshaw-Schulman syndrome (USS), a catastrophic phenotype of congenital thrombotic thrombocytopenic purpura, by analyzing 5 Japanese families. There were 3 mutations that occurred at exon-intron boundaries: 414+1G>A at intron 4, 686+1G>A at intron 6, a...

journal_title：Blood

authors： Matsumoto M,Kokame K,Soejima K,Miura M,Hayashi S,Fujii Y,Iwai A,Ito E,Tsuji Y,Takeda-Shitaka M,Iwadate M,Umeyama H,Yagi H,Ishizashi H,Banno F,Nakagaki T,Miyata T,Fujimura Y

更新日期：2004-02-15 00:00:00

abstract：:We have previously demonstrated a significant inverse correlation between circulating thrombopoietin (TPO) levels and peripheral platelet (PLT) counts in patients with thrombocytopenia secondary to megakaryocytic hypoplasia but not in patients with immune thrombocytopenic purpura (ITP; Chang et al, Blood 88:3354, 1996...

journal_title：Blood

authors： Chang M,Qian JX,Lee SM,Joubran J,Fernandez G,Nichols J,Knoppel A,Buzby JS

更新日期：1999-04-15 00:00:00

abstract：:Collagen binding to glycoprotein VI (GPVI) induces signals critical for platelet activation in thrombosis. Both ligand-induced GPVI signaling through its coassociated Fc-receptor gamma-chain (FcRgamma) immunoreceptor tyrosine-activation motif (ITAM) and the calmodulin inhibitor, W7, dissociate calmodulin from GPVI and...

journal_title：Blood

authors： Gardiner EE,Karunakaran D,Arthur JF,Mu FT,Powell MS,Baker RI,Hogarth PM,Kahn ML,Andrews RK,Berndt MC

更新日期：2008-01-01 00:00:00

abstract：:Some patients lose chimerism following nonmyeloablative hematopoietic cell transplantation (HCT), yet, surprisingly, enjoy sustained tumor remissions. We hypothesized that host-versus-graft (HVG) alloresponses might induce antitumor effects against recipient tumors. We explored this question in mice by administering r...

journal_title：Blood

authors： Rubio MT,Kim YM,Sachs T,Mapara M,Zhao G,Sykes M

更新日期：2003-09-15 00:00:00

abstract：:DNA methylation has pivotal regulatory roles in mammalian development, retrotransposon silencing, genomic imprinting, X-chromosome inactivation, and cancer. Cancer cells display highly dysregulated DNA methylation profiles, characterized by global hypomethylation in conjunction with hypermethylation of promoter CpG is...

journal_title：Blood

authors： Lio CJ,Yuita H,Rao A

更新日期：2019-10-31 00:00:00

abstract：:An abnormal von Willebrand factor (vWF) gene restriction pattern has been found in a patient with von Willebrand disease. Because this gene alteration is not present in his parents or in 50 normal and 25 affected subjects, and the restriction fragment length polymorphism haplotypes are inherited normally in the patien...

journal_title：Blood

authors： Bernardi F,Marchetti G,Guerra S,Casonato A,Gemmati D,Patracchini P,Ballerini G,Conconi F

更新日期：1990-02-01 00:00:00

abstract：:We evaluated the efficacy of umbilical cord blood (UCB) in the setting of a nonmyeloablative regimen consisting of fludarabine (200 mg/m2), cyclophosphamide (50 mg/kg), and a single fraction of total body irradiation (200 cGy) with cyclosporine and mycophenolate mofetil for posttransplantation immunoprophylaxis. The t...

journal_title：Blood

authors： Brunstein CG,Barker JN,Weisdorf DJ,DeFor TE,Miller JS,Blazar BR,McGlave PB,Wagner JE

更新日期：2007-10-15 00:00:00

abstract：:Emerging successful clinical data on gene therapy using adeno-associated viral (AAV) vector for hemophilia B (HB) showed that the risk of cellular immune response to vector capsid is clearly dose dependent. To decrease the vector dose, we explored AAV-8 (1-3 × 10(12) vg/kg) encoding a hyperfunctional factor IX (FIX-Pa...

journal_title：Blood

authors： Crudele JM,Finn JD,Siner JI,Martin NB,Niemeyer GP,Zhou S,Mingozzi F,Lothrop CD Jr,Arruda VR

更新日期：2015-03-05 00:00:00

abstract：:p38 MAP kinase in human platelets is activated by platelet agonists including thrombin, thromboxane A2 (TxA2), ADP, and others. However, both upstream mechanisms of p38 MAP kinase activation, and their downstream sequelae, are presently controversial and essentially unclear. Certain studies report sequential activatio...

journal_title：Blood

authors： Begonja AJ,Geiger J,Rukoyatkina N,Rauchfuss S,Gambaryan S,Walter U

更新日期：2007-01-15 00:00:00

abstract：:Ristocetin and vancomycin are structurally similar glycopeptide antibiotics. Both vancomycin and ristocetin in high concentrations (3.0 mg/ml) cause the precipitation of fibrinogen, plasminogen, and IgG from platelet-poor plasma (PPP). In contrast to ristocetin, vanomycin (0.5-1.5 mg/ml) does not agglutinate platelets...

journal_title：Blood

authors： Moake JL,Cimo PL,Peterson DM,Roper P,Natelson EA

更新日期：1977-09-01 00:00:00

abstract：:Down-regulation of immune responses by regulatory T (Treg) cells is an important mechanism involved in the induction of tolerance to allo-antigens (Ags). Recently, a novel subset of Ag-specific T-cell receptor (TCR)alpha beta+ CD4(-)CD8- (double-negative [DN]) Treg cells has been found to be able to prevent the reject...

journal_title：Blood

authors： Fischer K,Voelkl S,Heymann J,Przybylski GK,Mondal K,Laumer M,Kunz-Schughart L,Schmidt CA,Andreesen R,Mackensen A

更新日期：2005-04-01 00:00:00

abstract：:CXC ligand 12 (CXCL12; also known as stromal cell-derived factor 1alpha/SDF-1alpha) chemoattracts hematopoietic stem and progenitor cells (HSCs/HPCs) and is thought to play a crucial role in the mobilization of HSCs/HPCs from the bone marrow. CD26 (dipeptidylpeptidase IV [DPPIV]) is a membrane-bound extracellular pept...

journal_title：Blood

authors： Christopherson KW 2nd,Cooper S,Broxmeyer HE

更新日期：2003-06-15 00:00:00

abstract：:The International Immune Tolerance Study was a multicenter, prospective, randomized comparison of high-dose (HD; 200 IU/kg/d) and low-dose (LD; 50 IU/kg 3 times/week) factor VIII regimens in 115 "good-risk," severe high-titer inhibitor hemophilia A subjects. Sixty-six of 115 subjects reached the defined study end poin...

journal_title：Blood

authors： Hay CR,DiMichele DM,International Immune Tolerance Study.

更新日期：2012-02-09 00:00:00

abstract：:Pretreatment cytogenetics is a known predictor of outcome in hematologic malignancies. However, its usefulness in adult acute lymphoblastic leukemia (ALL) is generally limited to the presence of the Philadelphia (Ph) chromosome because of the low incidence of other recurrent abnormalities. We present centrally reviewe...

journal_title：Blood

authors： Moorman AV,Harrison CJ,Buck GA,Richards SM,Secker-Walker LM,Martineau M,Vance GH,Cherry AM,Higgins RR,Fielding AK,Foroni L,Paietta E,Tallman MS,Litzow MR,Wiernik PH,Rowe JM,Goldstone AH,Dewald GW,Adult Leukaemia Worki

更新日期：2007-04-15 00:00:00

abstract：:Approximately 4% to 5% of all bone marrow (BM) cells and 8% to 9% of low density BM cells from FVB/N and BALB/c mice (Ly-6a haplotype) show high to intermediate expression of Ly-6E.1 antigen, recognized by the Sca-1 antibody. Functional properties of enriched cells expressing Ly-6E.1-allelic form of Sca-1 antigen were...

journal_title：Blood

authors： Jurecic R,Van NT,Belmont JW

更新日期：1993-11-01 00:00:00

abstract：:It has been suggested that human cytomegalovirus (HCMV) evades the immune system by infecting and paralyzing antigen-presenting cells. This view is based mainly on studies of dendritic cells (DCs) obtained after culture of monocytes (moDCs). It is contradicted by the asymptomatic course of HCMV infection in healthy pe...

journal_title：Blood

authors： Kvale EØ,Dalgaard J,Lund-Johansen F,Rollag H,Farkas L,Midtvedt K,Jahnsen FL,Brinchmann JE,Olweus J

更新日期：2006-03-01 00:00:00

abstract：:Pharmaceutical preparations of normal human immunoglobulin (IgG) are known to contain high-avidity and neutralizing antibodies (Ab) to the cytokines interleukin (IL)-1alpha, IL-6, and interferon (IFN)alpha. To test for other cytokine Ab, 23 batches of IgG were tested for saturable binding to eight 125I-labeled recombi...

journal_title：Blood

authors： Svenson M,Hansen MB,Ross C,Diamant M,Rieneck K,Nielsen H,Bendtzen K

更新日期：1998-03-15 00:00:00

abstract：:The content of filamentous actin in individual platelets was measured by flow cytometry, using a fluorescent probe specific for filamentous actin (F-actin), 7-nitrobenz-2-oxa-1,3-phallacidin (NBD-phallacidin). NBD-phallacidin binding to fixed platelets was specific in that either pretreatment of platelets with unlabel...

journal_title：Blood

authors： Oda A,Daley JF,Cabral C,Kang JH,Smith M,Salzman EW

更新日期：1992-02-15 00:00:00

abstract：:Although NPM1 gene mutations leading to aberrant cytoplasmic expression of nucleophosmin (NPMc(+)) are the most frequent genetic lesions in acute myeloid leukemia, there is yet no experimental model demonstrating their oncogenicity in vivo. We report the generation and characterization of a transgenic mouse model expr...

journal_title：Blood

authors： Cheng K,Sportoletti P,Ito K,Clohessy JG,Teruya-Feldstein J,Kutok JL,Pandolfi PP

更新日期：2010-04-22 00:00:00

abstract：:Neutrophils were examined for their ability to recognize and ingest beads coated with amino-derivatives of glucose, mannose, and galactose. Radioactive or fluorescent beads coated with any of the three sugars were ingested to an extent three times that observed with albumin-coated beads. Enhancement of ingestion of su...

journal_title：Blood

authors： Doolittle RL,Packman CH,Lichtman MA

更新日期：1983-09-01 00:00:00

abstract：:Fetal hemoglobin (Hb F) production in sickle cell (SS) disease and in normal individuals varies over a 20-fold range and is under genetic control. Previous studies suggested that variant Hb F levels might be controlled by genetic loci separate from the beta-globin complex on chromosome 11. Using microscopic radial imm...

journal_title：Blood

authors： Dover GJ,Smith KD,Chang YC,Purvis S,Mays A,Meyers DA,Sheils C,Serjeant G

更新日期：1992-08-01 00:00:00

abstract：:The report of the so-called Berlin patient cured of HIV with hematopoietic stem cell transplantation and a few other studies raised tremendous hope, excitement, and curiosity in the field. The National Heart, Lung and Blood Institute of the National Institutes of Health convened a Working Group to address emerging hea...

journal_title：Blood

authors： Zou S,Glynn S,Kuritzkes D,Shah M,Cook N,Berliner N,NHLBI AIDS Blood Session Working Group.

更新日期：2013-10-31 00:00:00

abstract：:Antisense oligodeoxynucleotide (ODN) drugs might be more effective if their delivery was optimized and they were targeted to short-lived proteins encoded by messenger RNA (mRNA) species with equally short half-lives. To test this hypothesis, an ODN targeted to the c-myb proto-oncogene was developed and used to purge m...

journal_title：Blood

authors： Luger SM,O'Brien SG,Ratajczak J,Ratajczak MZ,Mick R,Stadtmauer EA,Nowell PC,Goldman JM,Gewirtz AM

更新日期：2002-02-15 00:00:00

abstract：:Hermansky-Pudlak syndrome (HPS) is an inherited hemorrhagic disease affecting the related subcellular organelles platelet dense granules, lysosomes, and melanosomes. The mouse genes for HPS, pale ear and pearl, orthologous to the human HPS1 and HPS2 (ADTB3A) genes, encode a novel protein of unknown function and the be...

journal_title：Blood

authors： Feng L,Novak EK,Hartnell LM,Bonifacino JS,Collinson LM,Swank RT

更新日期：2002-03-01 00:00:00

abstract：:CCR5 is a receptor for several beta chemokines and the entry coreceptor used by macrophage-tropic (R5) strains of HIV-1. In addition to supporting viral entry, CCR5 ligation by the HIV-1 envelope glycoprotein 120 (gp120) can activate intracellular signals in macrophages and trigger inflammatory mediator release. Using...

journal_title：Blood

authors： Tomkowicz B,Lee C,Ravyn V,Cheung R,Ptasznik A,Collman RG

更新日期：2006-08-15 00:00:00

abstract：:The calcium ionophore A23187 promotes histamine synthesis in murine bone marrow cells by increasing the expression of mRNA encoding histidine decarboxylase (HDC), the histamine-forming enzyme. The cells responsible for this biological activity copurify with hematopoietic progenitors in terms of density, light scatter ...

journal_title：Blood

authors： Dy M,Arnould A,Lemoine FM,Machavoine F,Ziltener H,Schneider E

更新日期：1996-04-15 00:00:00

abstract：:Leukocyte extravasation involves interdependent signaling pathways underlying the complex dynamics of firm adhesion, crawling, and diapedesis. While signal transduction by agonist-bound chemokine receptors plays a central role in the above responses, it is unclear how it contributes to the sustained and concurrent nat...

journal_title：Blood

authors： Molteni R,Crespo CL,Feigelson S,Moser C,Fabbri M,Grabovsky V,Krombach F,Laudanna C,Alon R,Pardi R

更新日期：2009-07-30 00:00:00

abstract：:Phosphoinositide 3-kinase gamma (PI3Kgamma) in neutrophils plays a critical role in the directed migration of these cells into inflamed tissues. In this study, we demonstrate the importance of the endothelial component of PI3Kgamma activity relative to its leukocyte counterpart in supporting neutrophil interactions wi...

journal_title：Blood

authors： Puri KD,Doggett TA,Huang CY,Douangpanya J,Hayflick JS,Turner M,Penninger J,Diacovo TG

更新日期：2005-07-01 00:00:00

abstract：:To determine whether the erythropoietin receptor (Epo-R) plays a role in the course of malignant erythropoietic disorders, this gene was studied in murine and human erythroleukemia cells. An altered Epo-R gene was found in a murine Friend erythroleukemia cell line, FCL1, due to a spleen focus-forming virus (SFFV) long...

journal_title：Blood

authors： Chretien S,Moreau-Gachelin F,Apiou F,Courtois G,Mayeux P,Dutrillaux B,Cartron JP,Gisselbrecht S,Lacombe C

更新日期：1994-04-01 00:00:00

abstract：:A 42-yr-old woman with systemic lupus erythematosus without bleeding diathesis developed a prolonged activated partial thromboplastin time that was not corrected by normal plasma. An inhibitor that acted rapidly and inactivated 0.5 U/ml plasma thromboplastin antecedent (PTA, factor XI) at a 1:200 plasma dilution was d...

journal_title：Blood

authors： Poon MC,Saito H,Koopman WJ

更新日期：1984-06-01 00:00:00