MST1 mutations in autosomal recessive primary immunodeficiency characterized by defective naive T-cell survival.

abstract：:To characterize and clarify the function of CD34 antigen experimentally, we isolated two types of CD34 mRNA from a cDNA library of murine stromal cell line, PA-6 stimulated with lipopolysaccharide (LPS) and 12-o-tetra-decanoylphorbol 13-acetate (TPA) using a human CD34 probe. In addition to the clone (open reading fra...

journal_title：Blood

authors： Suda J,Sudo T,Ito M,Ohno N,Yamaguchi Y,Suda T

更新日期：1992-05-01 00:00:00

abstract：:Between February 1996 and December 2004, the German Leukemia Study Initiative registered 1766 consecutive patients for the acute myeloid leukemia (AML) 96 study, all of whom were diagnosed by central cytomorphology according to the French-American-British (FAB) and the new World Health Organization (WHO) classificatio...

journal_title：Blood

authors： Wandt H,Schäkel U,Kroschinsky F,Prange-Krex G,Mohr B,Thiede C,Pascheberg U,Soucek S,Schaich M,Ehninger G

更新日期：2008-02-15 00:00:00

abstract：:Chronic active EBV disease (CAEBV) is a lymphoproliferative disorder characterized by markedly elevated levels of antibody to EBV or EBV DNA in the blood and EBV RNA or protein in lymphocytes in tissues. We present our experience with CAEBV during the last 28 years, including the first 8 cases treated with hematopoiet...

journal_title：Blood

authors： Cohen JI,Jaffe ES,Dale JK,Pittaluga S,Heslop HE,Rooney CM,Gottschalk S,Bollard CM,Rao VK,Marques A,Burbelo PD,Turk SP,Fulton R,Wayne AS,Little RF,Cairo MS,El-Mallawany NK,Fowler D,Sportes C,Bishop MR,Wilson W,St

更新日期：2011-06-02 00:00:00

abstract：:Activation of endothelial cells, important in processes such as angiogenesis, is regulated by cell surface receptors, including those in the tyrosine kinase (RTK) family. Receptor activity, in turn, can be modulated by phosphorylation, turnover, or proteolytic release of a soluble extracellular domain. Previously, we ...

journal_title：Blood

authors： Yabkowitz R,Meyer S,Black T,Elliott G,Merewether LA,Yamane HK

更新日期：1999-03-15 00:00:00

abstract：:To increase transgenic production of granulocyte-macrophage colony-stimulating factor (GM-CSF), we mutated the mRNA's 3'-untranslated region, AUUUA instability elements. Expression vectors containing human or murine GM-CSF cDNAs coding for wild-type (GM-AUUUA) or mutant versions with reiterated AUGUA repeats (GM-AUGUA...

journal_title：Blood

authors： Rajagopalan LE,Burkholder JK,Turner J,Culp J,Yang NS,Malter JS

更新日期：1995-10-01 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

authors： Chen J,Zhou H,Diacovo A,Zheng XL,Emsley J,Diacovo TG

更新日期：2014-12-11 00:00:00

abstract：:The ultrastructure of the lymphocytes from three children with severe combined immunodeficiency disease (SCID) is described. Parallel tubular arrays (PTA) were found in a large percentage of circulating lymphocytes (53%, 41%, and 13%) in three SCID patients when compared to age-matched controls. The size of these incl...

journal_title：Blood

authors： Payne CM,Jones JF,Sieber OF Jr,Fulginiti VA

更新日期：1977-07-01 00:00:00

abstract：:Recent evidence suggests chronic myeloid leukemia (CML) stem cells are insensitive to kinase inhibitors and responsible for minimal residual disease in treated patients. We investigated whether CML stem cells, in a transgenic mouse model of CML-like disease or derived from patients, are dependent on Bcr-Abl. In the tr...

journal_title：Blood

authors： Hamilton A,Helgason GV,Schemionek M,Zhang B,Myssina S,Allan EK,Nicolini FE,Müller-Tidow C,Bhatia R,Brunton VG,Koschmieder S,Holyoake TL

更新日期：2012-02-09 00:00:00

abstract：:The expression of Pax5 commits common lymphoid progenitor cells to B-lymphoid lineage differentiation. Little is known of possible variations in the levels of Pax5 expression and their influences on hematopoietic development. We have developed a retroviral transduction system that allows for the study of possible inte...

journal_title：Blood

authors： Simmons S,Knoll M,Drewell C,Wolf I,Mollenkopf HJ,Bouquet C,Melchers F

更新日期：2012-11-01 00:00:00

abstract：:A primary function of the World Marrow Donor Association is to establish general guidelines covering collaboration between international donor registries and practice in regard to bone marrow transplants (BMTs) in which the donor and recipient reside in different countries. To this end, a special report proposing spec...

journal_title：Blood

authors： Goldman JM

更新日期：1994-11-01 00:00:00

abstract：:Intravascular hemolysis describes the relocalization of heme and hemoglobin (Hb) from erythrocytes to plasma. We investigated the concept that erythrocyte membrane microparticles (MPs) concentrate cell-free heme in human hemolytic diseases, and that heme-laden MPs have a physiopathological impact. Up to one-third of c...

journal_title：Blood

authors： Camus SM,De Moraes JA,Bonnin P,Abbyad P,Le Jeune S,Lionnet F,Loufrani L,Grimaud L,Lambry JC,Charue D,Kiger L,Renard JM,Larroque C,Le Clésiau H,Tedgui A,Bruneval P,Barja-Fidalgo C,Alexandrou A,Tharaux PL,Boulanger CM

更新日期：2015-06-11 00:00:00

abstract：:Sympathetic axons embedded in a few arterioles and vasa vasora were recently shown to store tissue plasminogen activator (t-PA) in vesicles. But the extension of such t-PA axons to arteries and arterioles throughout the organism has not been verified. Confirmation of this anatomy would identify a second significant so...

journal_title：Blood

authors： Hao Z,Guo C,Jiang X,Krueger S,Pietri T,Dufour S,Cone RE,O'Rourke J

更新日期：2006-07-01 00:00:00

abstract：:A myeloma cell-reactive monoclonal antibody (MoAb), MM4, was generated from BALB/c mice immunized with alternate injections of cells from two human multiple myeloma (MM) cell lines. Screening by the enzyme-linked immunosorbent assay (ELISA) technique showed that MM4 reacted with human MM cell lines (7 of 7 positive), ...

journal_title：Blood

authors： Tong AW,Lee JC,Stone MJ

更新日期：1987-01-01 00:00:00

abstract：:Genetic alteration is considered a probable cause of malignant lymphoma. Folate and methionine metabolism play essential roles in DNA synthesis and DNA methylation, and their metabolic pathways might thus affect disease susceptibility. In the present study, 2 polymorphisms were evaluated for a folate metabolic enzyme,...

journal_title：Blood

authors： Matsuo K,Suzuki R,Hamajima N,Ogura M,Kagami Y,Taji H,Kondoh E,Maeda S,Asakura S,Kaba S,Nakamura S,Seto M,Morishima Y,Tajima K

更新日期：2001-05-15 00:00:00

abstract：:Ectopic expression of mutated K-ras or N-ras in the interleukin 6 (IL-6)-dependent ANBL6 multiple myeloma cell line induces cytokine-independent growth. To investigate the signaling pathways activated by oncogenic ras that may stimulate IL-6-independent growth, we compared ANBL6 cells stably transfected with mutated K...

journal_title：Blood

authors： Hu L,Shi Y,Hsu JH,Gera J,Van Ness B,Lichtenstein A

更新日期：2003-04-15 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：:Allogeneic hematopoietic stem cell transplantation (alloHSCT) is the only potentially curative treatment available for patients with B-cell chronic lymphocytic leukemia (B-CLL). Here, we show that post-alloHSCT antibody repertoires can be mined for the discovery of fully human monoclonal antibodies to B-CLL cell-surfa...

journal_title：Blood

authors： Baskar S,Suschak JM,Samija I,Srinivasan R,Childs RW,Pavletic SZ,Bishop MR,Rader C

更新日期：2009-11-12 00:00:00

abstract：:A study in 121 infants with severe combined immunodeficiency (SCID) was performed to determine the prevalence of an engraftment by transplacentally acquired maternal T cells and to explore clinical and immunological findings related to this abnormality. Each newly diagnosed patient with SCID presenting with circulatin...

journal_title：Blood

authors： Müller SM,Ege M,Pottharst A,Schulz AS,Schwarz K,Friedrich W

更新日期：2001-09-15 00:00:00

abstract：:We investigated the effects of granulocyte colony-stimulating factor (G-CSF) on cytokine and cytokine receptor plasma levels and on the in vivo host response to Salmonella abortus equi endotoxin in healthy males. Twenty volunteers received 0.8 ng/kg endotoxin and saline intravenously 1 week apart in randomized order. ...

journal_title：Blood

authors： Pollmächer T,Korth C,Mullington J,Schreiber W,Sauer J,Vedder H,Galanos C,Holsboer F

更新日期：1996-02-01 00:00:00

abstract：:Serum cobalamin (vitamin B12) and unsaturated B12 binding capacity (UBBC) have been measured in 24 cases of hypereosinophilia: 16 were cases of hypereosinophilic syndrome (HES) and 8 of secondary eosinophilia. The two groups were similar with respect to absolute eosinophil counts. Serum cobalamin and UBBC were found t...

journal_title：Blood

authors： Zittoun J,Farcet JP,Marquet J,Sultan C,Zittoun R

更新日期：1984-04-01 00:00:00

abstract：:We have re-evaluated the previously reported ability of TLCK-thrombin (N alpha-tosyl-L-lysine chloromethyl ketone-treated alpha-thrombin) and PPACK-thrombin (D-phenylalanyl-L-prolyl-L-arginine chloromethyl ketone-treated alpha-thrombin) to inhibit alpha-thrombin-induced platelet activation (Harmon JT, Jamieson GA: J B...

journal_title：Blood

authors： Greco NJ,Tenner TE Jr,Tandon NN,Jamieson GA

更新日期：1990-05-15 00:00:00

abstract：:Dendritic cells (DCs) are specialized antigen-presenting cells that monitor the antigenic environment and activate naive T cells. The role of DCs is not only to sense danger but also to tolerize the immune system to antigens encountered in the absence of maturation/inflammatory stimuli. Indeed, if a naive T cell encou...

journal_title：Blood

authors： Levings MK,Gregori S,Tresoldi E,Cazzaniga S,Bonini C,Roncarolo MG

更新日期：2005-02-01 00:00:00

abstract：:The biologic effects of endothelin-1 (ET-1) are not limited to its potent vasoconstricting activity. The endothelin receptors, ETA and ETB, have differential tissue and functional distributions. Here we showed that dendritic cells (DCs), the major antigen-presenting cells in the adaptive limb of the immune system, pro...

journal_title：Blood

authors： Guruli G,Pflug BR,Pecher S,Makarenkova V,Shurin MR,Nelson JB

更新日期：2004-10-01 00:00:00

abstract：:Forty-nine patients with primary myelodysplastic syndromes (MDS) were subclassified according to French-American-British (FAB) Cooperative Group criteria. Eight patients had acquired idiopathic sideroblastic anemia (AISA), ten had chronic myelomonocytic leukemia (CMMoL), 14 had refractory anemia (RA), nine had refract...

journal_title：Blood

authors： Jacobs RH,Cornbleet MA,Vardiman JW,Larson RA,Le Beau MM,Rowley JD

更新日期：1986-06-01 00:00:00

abstract：:Growth of human B-cell precursors (BCP) was achieved by plating fetal CD10+ surface-mu (s mu)- cells in liquid medium onto bone marrow-derived fibroblastic stromal cell layers deprived of hematopoietic cells. Proliferation of the fetal BCP was strongly potentiated by the addition of interleukin-7 (IL-7) to the culture...

journal_title：Blood

authors： Moreau I,Duvert V,Banchereau J,Saeland S

更新日期：1993-03-01 00:00:00

abstract：:Essential thrombocythemia (ET) is characterized by abnormal megakaryopoiesis and enhanced thrombotic risk. Once-daily low-dose aspirin is the recommended antithrombotic regimen, but accelerated platelet generation may reduce the duration of platelet cyclooxygenase-1 (COX-1) inhibition. We performed a multicenter doubl...

journal_title：Blood

authors： Rocca B,Tosetto A,Betti S,Soldati D,Petrucci G,Rossi E,Timillero A,Cavalca V,Porro B,Iurlo A,Cattaneo D,Bucelli C,Dragani A,Di Ianni M,Ranalli P,Palandri F,Vianelli N,Beggiato E,Lanzarone G,Ruggeri M,Carli G,Ell

更新日期：2020-07-09 00:00:00

abstract：:Human transferrin receptor-2 (TFR-2) is a protein highly homologous to TFR-1/CD71 and is endowed with the ability to bind transferrin (TF) with low affinity. High levels of TFR-2 mRNA were found in the liver and in erythroid precursors. Mutations affecting the TFR-2 gene led to hemochromatosis type 3, a form of inheri...

journal_title：Blood

authors： Deaglio S,Capobianco A,Calì A,Bellora F,Alberti F,Righi L,Sapino A,Camaschella C,Malavasi F

更新日期：2002-11-15 00:00:00

abstract：:Protein S, an inhibitor of coagulation, interacts reversibly with C4b binding protein (C4bBP). The physiologic role of this complex formation remains unknown. In this study we examined the possibility that protein S would facilitate C4bBP binding to the surface of neutrophils where, in turn, this complex could help pr...

journal_title：Blood

authors： Furmaniak-Kazmierczak E,Hu CY,Esmon CT

更新日期：1993-01-15 00:00:00

abstract：:Clonal expansion of activated T cells is controlled by homeostatic mechanisms leading to cell death of a large proportion of the cells. The CD3/TcR pathway induces cell death, mostly when triggered in the absence of costimulatory signal. The unique T cell-specific chemokine of the C class, lymphotactin (Lptn), has rec...

journal_title：Blood

authors： Cerdan C,Devilard E,Xerri L,Olive D

更新日期：2001-04-15 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