Parallel tubular arrays in severe combined immunodeficiency disease: an ultrastructural study of peripheral blood lymphocytes.

abstract：:Bleeding problems are associated with defects in platelet alpha-granules, yet little is known about how these granules are formed and released. Mutations affecting VPS33B, a novel Sec1/Munc18 protein, have recently been linked to arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome. We have characterized ...

journal_title：Blood

authors： Lo B,Li L,Gissen P,Christensen H,McKiernan PJ,Ye C,Abdelhaleem M,Hayes JA,Williams MD,Chitayat D,Kahr WH

更新日期：2005-12-15 00:00:00

abstract：:Colony forming cells (CFC) with high proliferative potential have been detected in nutrient agar cultures of human bone marrow cells containing recombinant human interleukin-3 (IL-3) and granulocyte macrophage colony stimulating factor (GM-CSF). These CFC were detected by the formation of large colonies with diameters...

journal_title：Blood

authors： McNiece IK,Stewart FM,Deacon DM,Temeles DS,Zsebo KM,Clark SC,Quesenberry PJ

更新日期：1989-08-01 00:00:00

abstract：:Therapeutic preparations of polyspecific IgG obtained from plasma pools of a large number of normal donors (IVIg) express anti-idiotypic activity against a wide spectrum of natural and disease-associated autoantibodies. The present study investigated the origin of anti-idiotypic activity against autoantibodies to fact...

journal_title：Blood

authors： Dietrich G,Algiman M,Sultan Y,Nydegger UE,Kazatchkine MD

更新日期：1992-06-01 00:00:00

abstract：:A1, a bcl-2 family member, has been identified as a hematopoietic-specific, early inducible gene. In this study it is shown that stable transfection of A1 into an interleukin-3 (IL-3)-dependent myeloid precursor cell line, 32D c13, leads to a retardation of IL-3 withdrawal-induced cell death similar to that observed w...

journal_title：Blood

authors： Lin EY,Orlofsky A,Wang HG,Reed JC,Prystowsky MB

更新日期：1996-02-01 00:00:00

abstract：:Germline mutations of the CDKN2A (p16(INK4A)) tumor suppressor gene predispose patients to melanoma and pancreatic carcinoma. In contrast, mutations of the murine CDKN2A gene predispose BALB/c mice to pristane-induced plasmacytoma. We describe here a family in which a germline mutation of CDKN2A is present in 4 indivi...

journal_title：Blood

authors： Dilworth D,Liu L,Stewart AK,Berenson JR,Lassam N,Hogg D

更新日期：2000-03-01 00:00:00

abstract：:Primary cutaneous lymphomas (PCLs) are clonal T- or B-cell neoplasms, which originate in the skin. In recent years, mast cells were described as regulators of the tumor microenvironment in different human malignancies. Here, we investigated the role of mast cells in the tumor microenvironment of PCL. We found signific...

journal_title：Blood

authors： Rabenhorst A,Schlaak M,Heukamp LC,Förster A,Theurich S,von Bergwelt-Baildon M,Büttner R,Kurschat P,Mauch C,Roers A,Hartmann K

更新日期：2012-09-06 00:00:00

abstract：:Erythrocytes of neonates and adults were incubated with increasing concentrations of H2O2 in the presence of a catalase inhibitor and in the absence of glucose; the pattern of oxidation of vitamin E was analyzed in relationship to that of glutathione, hemoglobin, and polyunsaturated fatty acids (PUFA), and in relation...

journal_title：Blood

authors： Vanderpas J,Vertongen F

更新日期：1985-12-01 00:00:00

abstract：:An unmet need in cell engineering is the availability of a single transgene encoded, functionally inert, human polypeptide that can serve multiple purposes, including ex vivo cell selection, in vivo cell tracking, and as a target for in vivo cell ablation. Here we describe a truncated human EGFR polypeptide (huEGFRt) ...

journal_title：Blood

authors： Wang X,Chang WC,Wong CW,Colcher D,Sherman M,Ostberg JR,Forman SJ,Riddell SR,Jensen MC

更新日期：2011-08-04 00:00:00

abstract：:We have retrospectively assessed the neurological manifestations in 34 patients with hemophagocytic lymphohistiocytosis (HLH) in a single center. Clinical, radiological, and cerebrospinal fluid (CSF) cytology data were analyzed according to treatment modalities. Twenty-five patients (73%) had evidence of central nervo...

journal_title：Blood

authors： Haddad E,Sulis ML,Jabado N,Blanche S,Fischer A,Tardieu M

更新日期：1997-02-01 00:00:00

abstract：:Erythropoietin (Epo) and dimethyl sulfoxide (DMSO) are believed to induce the differentiation of transformed erythroid cells by different signal transduction pathways. We have now obtained evidence for the interaction of these pathways. We used a Rauscher murine erythroleukemia cell line with a relatively low (8% to 1...

journal_title：Blood

authors： Chern Y,Yonekura S,Sytkowski AJ

更新日期：1990-12-01 00:00:00

abstract：:Proteolytic events at the cell surface are essential in the regulation of signal transduction pathways. During the past years, the family of type II transmembrane serine proteases (TTSPs) has acquired an increasing relevance because of their privileged localization at the cell surface, although our current understandi...

journal_title：Blood

authors： Folgueras AR,de Lara FM,Pendás AM,Garabaya C,Rodríguez F,Astudillo A,Bernal T,Cabanillas R,López-Otín C,Velasco G

更新日期：2008-09-15 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：:Congenital hypothyroidism (CH) is one of the most prevalent endocrine diseases, for which the underlying mechanisms remain unknown; it is often accompanied by anemia and immunodeficiency in patients. Here, we created a severe CH model together with anemia and T lymphopenia to mimic the clinical features of hypothyroid...

journal_title：Blood

authors： Zhang Y,Xue Y,Cao C,Huang J,Hong Q,Hai T,Jia Q,Wang X,Qin G,Yao J,Wang X,Zheng Q,Zhang R,Li Y,Luo A,Zhang N,Shi G,Wang Y,Ying H,Liu Z,Wang H,Meng A,Zhou Q,Wei H,Liu F,Zhao J

更新日期：2017-11-16 00:00:00

abstract：:Global gene expression profiling of highly purified 5q-deleted CD34+CD38(-)Thy1+ cells in 5q- myelodysplastic syndromes (MDSs) supported that they might originate from and outcompete normal CD34+CD38(-)Thy1+ hematopoietic stem cells. Few but distinct differences in gene expression distinguished MDS and normal stem cel...

journal_title：Blood

authors： Nilsson L,Edén P,Olsson E,Månsson R,Astrand-Grundström I,Strömbeck B,Theilgaard-Mönch K,Anderson K,Hast R,Hellström-Lindberg E,Samuelsson J,Bergh G,Nerlov C,Johansson B,Sigvardsson M,Borg A,Jacobsen SE

更新日期：2007-10-15 00:00:00

abstract：:Over the years, methods of cytogenetic analysis evolved and became part of routine laboratory testing, providing valuable diagnostic and prognostic information in hematologic disorders. Karyotypic aberrations contribute to the understanding of the molecular pathogenesis of disease and thereby to rational application o...

journal_title：Blood

authors： Maciejewski JP,Mufti GJ

更新日期：2008-08-15 00:00:00

abstract：:The thrombolytic efficacy of recombinant unglycosylated full length single chain urokinase-type plasminogen activator (rscu-PA, saruplase), applied either as single intravenous bolus or as a continuous infusion over 60 minutes, was studied in 5 randomized blinded groups of 5 dogs with combined copper coil induced coro...

journal_title：Blood

authors： Rapold HJ,Wu ZM,Stassen T,Van de Werf F,Collen D

更新日期：1990-10-15 00:00:00

abstract：:Regulated by histone acetyltransferases and deacetylases (HDACs), histone acetylation is a key epigenetic mechanism controlling chromatin structure, DNA accessibility, and gene expression. HDAC inhibitors induce growth arrest, differentiation, and apoptosis of tumor cells and are used as anticancer agents. Here we des...

journal_title：Blood

authors： Roger T,Lugrin J,Le Roy D,Goy G,Mombelli M,Koessler T,Ding XC,Chanson AL,Reymond MK,Miconnet I,Schrenzel J,François P,Calandra T

更新日期：2011-01-27 00:00:00

abstract：:Glanzmann's thrombasthenia is an inherited bleeding disorder characterized by abnormalities of platelet membrane glycoproteins (GP) IIb and IIIa. Most patients, usually designated as type I, have been reported to have undetectable levels of GP IIb and GP IIIa with the assay used. We have used polyclonal rabbit antibod...

journal_title：Blood

authors： Nurden AT,Didry D,Kieffer N,McEver RP

更新日期：1985-04-01 00:00:00

abstract：:The mechanisms by which tumors metastasize to sentinel and distant lymph nodes, and beyond, are poorly understood. We developed transgenic mice that overexpress vascular endothelial growth factor-C (VEGF-C) and green fluorescent protein specifically in the skin and studied the effects of chemically-induced skin carcin...

journal_title：Blood

authors： Hirakawa S,Brown LF,Kodama S,Paavonen K,Alitalo K,Detmar M

更新日期：2007-02-01 00:00:00

abstract：:Rabbit platelets were aggregated by adenosine diphosphate (ADP), allowed to deaggregate and then separated into density subpopulations by centrifugation through discontinuous Stractan density gradients. Although ADP causes little or no release of the contents of the amine storage granules of rabbit platelets, ADP caus...

journal_title：Blood

authors： Packham MA,Perry DW,Kinlough-Rathbone RL,Rand ML,Guccione MA,Evans RM,Mustard JF

更新日期：1985-03-01 00:00:00

abstract：:The urokinase receptor (uPAR) plays an important role in regulation of fibronolysis, cell migration, and adhesion. In this study, we examined whether uPAR plays a role in modulating efferocytosis of neutrophils. Macrophages from uPAR(-/-) mice demonstrated enhanced ability to engulf viable wild-type (WT) neutrophils i...

journal_title：Blood

authors： Park YJ,Liu G,Tsuruta Y,Lorne E,Abraham E

更新日期：2009-07-23 00:00:00

abstract：:The regulation of megakaryocytopoiesis and thrombopoiesis appears to be under the control of an array of hematopoietic growth factors. To determine the relationship of endogenous thrombopoietic cytokine levels and circulating platelet (PLT) counts, we measured the levels of thrombo-poietin (TPO), interleukin-11 (IL-11...

journal_title：Blood

authors： Chang M,Suen Y,Meng G,Buzby JS,Bussel J,Shen V,van de Ven C,Cairo MS

更新日期：1996-11-01 00:00:00

abstract：:Fanconi anemia (FA) is an autosomal recessive disease characterized by congenital anomalies, aplastic anemia, and a susceptibility to leukemia. There are at least 8 complementation groups (A through H). Extensive analyses of the FA group C gene FANCC in Western countries revealed that 10% to 15% of FA patients have mu...

journal_title：Blood

authors： Futaki M,Yamashita T,Yagasaki H,Toda T,Yabe M,Kato S,Asano S,Nakahata T

更新日期：2000-02-15 00:00:00

abstract：:Bcl-2 protein expression has been associated with poor prognosis in patients with noncutaneous diffuse large B-cell lymphomas. In primary cutaneous large B-cell lymphomas, the location on the leg, the round-cell morphology defined as the predominance of centroblasts and immunoblasts over large centrocytes, and multipl...

journal_title：Blood

authors： Grange F,Petrella T,Beylot-Barry M,Joly P,D'Incan M,Delaunay M,Machet L,Avril MF,Dalac S,Bernard P,Carlotti A,Esteve E,Vergier B,Dechelotte P,Cassagnau E,Courville P,Saiag P,Laroche L,Bagot M,Wechsler J

更新日期：2004-05-15 00:00:00

abstract：:In this issue of Blood, Bhatia et al demonstrate the critical importance of an adherence to oral chemotherapy regimens in attaining cure for children with acute lymphoblastic leukemia (ALL) and identify race-specific determinants of adherence. ...

journal_title：Blood

authors： Winick N

更新日期：2014-10-09 00:00:00

abstract：:Humans and grey collie dogs with cyclic neutropenia are known to suffer from an increased rate of bacterial infection. Because of the previously described microanatomic abnormalities of lysosome formation found in the polymorphonuclear leukocytes (PMNs) of dogs with canine cyclic neutropenia, studies of these cells we...

journal_title：Blood

authors： Chusid MJ,Bujak JS,Dale DC

更新日期：1975-12-01 00:00:00

abstract：:Hereditary hemochromatosis is caused by mutations in the hereditary hemochromatosis protein (HFE), transferrin-receptor 2 (TfR2), hemojuvelin, hepcidin, or ferroportin genes. Hepcidin is a key iron regulator, which is secreted by the liver, and decreases serum iron levels by causing the down-regulation of the iron tra...

journal_title：Blood

authors： Gao J,Chen J,De Domenico I,Koeller DM,Harding CO,Fleming RE,Koeberl DD,Enns CA

更新日期：2010-04-22 00:00:00

abstract：:Lymphoproliferative disorders involving Epstein-Barr virus (EBV) infected natural killer (NK) cells are reported with increasing frequency, but the nature and role of EBV infection in these cells remains undefined. In this study, we have investigated virus-cell interactions in the EBV-positive YTN10 cell line, an NK-l...

journal_title：Blood

authors： Kanegane H,Wang F,Tosato G

更新日期：1996-12-15 00:00:00

abstract：:Retroviral gene therapy can restore immunity to infants with X-linked severe combined immunodeficiency (XSCID) caused by mutations in the IL2RG gene encoding the common gamma chain (gammac) of receptors for interleukins 2 (IL-2), -4, -7, -9, -15, and -21. We investigated the safety and efficacy of gene therapy as salv...

journal_title：Blood

authors： Chinen J,Davis J,De Ravin SS,Hay BN,Hsu AP,Linton GF,Naumann N,Nomicos EY,Silvin C,Ulrick J,Whiting-Theobald NL,Malech HL,Puck JM

更新日期：2007-07-01 00:00:00

abstract：:We studied the respiration and glucose utilization of suspensions of cultured lymphocytes. Respiration was measured by a couloximetric method using closed systems without a gas-suspension interface. The sensitivity of the method allowed measurements during a time interval of less than 1 hr. We found that the respirati...

journal_title：Blood

authors： Sand T,Condie R,Rosenberg A

更新日期：1977-08-01 00:00:00