Montreal platelet syndrome: a defect in calcium-activated neutral proteinase (calpain).

abstract：:In systemic light-chain amyloidosis, λ light chains produced by clonal plasma cells cause organ damage and early death. In pursuit of novel therapy, we developed 1 pool of short interfering RNA (siRNA) targeting the constant region of λ light chains that substantially and promptly reduces λ-light-chain production and ...

journal_title：Blood

authors： Zhou P,Ma X,Iyer L,Chaulagain C,Comenzo RL

更新日期：2014-05-29 00:00:00

abstract：:Little is known about the behavior of hematopoietic stem cells (HSCs) in primates because direct observations and competitive-repopulation assays are not feasible. Therefore, we used 2 different and independent experimental strategies, the tracking of transgene expression after retroviral-mediated gene transfer (N = 1...

journal_title：Blood

authors： Shepherd BE,Kiem HP,Lansdorp PM,Dunbar CE,Aubert G,LaRochelle A,Seggewiss R,Guttorp P,Abkowitz JL

更新日期：2007-09-15 00:00:00

abstract：:It has been hypothesized that some of the irreversible microrheologic abnormalities of sickle red blood cell (RBC) membranes could result from autoxidative perturbation. To model this possibility, we used micromechanical manipulation to examine the static extensional rigidity and inelastic or plastic behavior of norma...

journal_title：Blood

authors： Hebbel RP,Leung A,Mohandas N

更新日期：1990-09-01 00:00:00

abstract：:The transforming growth factor-beta (TGF-beta) signaling pathway is an essential regulator of cellular processes, including proliferation, differentiation, migration, and cell survival. During hematopoiesis, the TGF-beta signaling pathway is a potent negative regulator of proliferation while stimulating differentiatio...

journal_title：Blood

authors： Dong M,Blobe GC

更新日期：2006-06-15 00:00:00

abstract：:HTLV-II has been found in some cases of the rare T-cell form of hairy-cell leukemia (HCL) and in a leukopenic chronic T-cell leukemia mimicking HCL. We asked whether the virus is implicated in the more frequent B-cell form of HCL. DNA extracted from the mononuclear cells derived from spleen (eight cases) or peripheral...

journal_title：Blood

authors： Lion T,Razvi N,Golomb HM,Brownstein RH

更新日期：1988-10-01 00:00:00

abstract：:Dentin matrix protein 1 (DMP1) is a member of the small integrin-binding ligand N-linked glycoprotein (SIBLING) family, a group of proteins initially described as mineralized extracellular matrices components. More recently, SIBLINGs have been implicated in several key steps of cancer progression, including angiogenes...

journal_title：Blood

authors： Pirotte S,Lamour V,Lambert V,Alvarez Gonzalez ML,Ormenese S,Noël A,Mottet D,Castronovo V,Bellahcène A

更新日期：2011-02-24 00:00:00

abstract：:Transcriptional control has been identified as a key mechanism regulating the formation and subsequent behavior of hematopoietic stem cells. We have used a comparative genomics approach to identify transcriptional regulatory elements of the LMO2 gene, a transcriptional cofactor originally identified through its involv...

journal_title：Blood

authors： Landry JR,Kinston S,Knezevic K,Donaldson IJ,Green AR,Göttgens B

更新日期：2005-10-15 00:00:00

abstract：:Von Willebrand disease (VWD)-type 2B originates from a gain-of-function mutation in von Willebrand factor (VWF), resulting in enhanced platelet binding. Clinical manifestations include increased bleeding tendency, loss of large multimers, thrombocytopenia, and circulating platelet aggregates. We developed a mouse mode...

journal_title：Blood

authors： Rayes J,Hollestelle MJ,Legendre P,Marx I,de Groot PG,Christophe OD,Lenting PJ,Denis CV

更新日期：2010-06-10 00:00:00

abstract：:Acute promyelocytic leukemias (APLs) are characterized by a translocation that involves chromosomes 15 and 17. The translocation breakpoints have recently been identified and shown to involve the RAR-alpha gene on 17 and myl on 15. Here we report Southern blotting analysis of 26 APLs, including cases with normal karyo...

journal_title：Blood

authors： Biondi A,Rambaldi A,Alcalay M,Pandolfi PP,Lo Coco F,Diverio D,Rossi V,Mencarelli A,Longo L,Zangrilli D

更新日期：1991-04-01 00:00:00

abstract：:Activation of Janus tyrosine kinases (Jak) and Signal transducers and activators of transcription (Stat) after ligation of major histocompatibility complex class I (MHC-I) was explored in Jurkat T cells. Cross-linking of MHC-I mediated tyrosine phosphorylation of Tyk2, but not Jak1, Jak2, and Jak3. In addition, the tr...

journal_title：Blood

authors： Skov S,Nielsen M,Bregenholt S,Odum N,Claesson MH

更新日期：1998-05-15 00:00:00

abstract：:Dendritic cells (DCs) are considered critical for the induction of graft-versus-host disease (GVHD) after bone marrow transplantation (BMT). In addition to their priming function, dendritic cells have been shown to induce organ-tropism through induction of specific homing molecules on T cells. Using adoptive transfer ...

journal_title：Blood

authors： Kim TD,Terwey TH,Zakrzewski JL,Suh D,Kochman AA,Chen ME,King CG,Borsotti C,Grubin J,Smith OM,Heller G,Liu C,Murphy GF,Alpdogan O,van den Brink MR

更新日期：2008-03-01 00:00:00

abstract：:Human erythroid alpha-spectrin alleles responsible for hereditary elliptocytosis (alphaHE alleles) undergo increased incorporation into red blood cell membranes when the polymorphism alphaLELY (LELY: Low Expression LYon) occurs in trans. The alphaLELY polymorphism is characterized by a mutation in exon 40 at codon 185...

journal_title：Blood

authors： Wilmotte R,Harper SL,Ursitti JA,Maréchal J,Delaunay J,Speicher DW

更新日期：1997-11-15 00:00:00

abstract：:Cholesterol, in addition to providing rigidity to the fluid membrane, plays a critical role in receptor function, endocytosis, recycling, and signal transduction. In the present study, we examined the effect of membrane cholesterol on functional expression of tissue factor (TF), a cellular receptor for clotting factor...

journal_title：Blood

authors： Mandal SK,Iakhiaev A,Pendurthi UR,Rao LV

更新日期：2005-01-01 00:00:00

abstract：:Thrombopoietin (TPO) is a hematopoietic growth factor that regulates megakaryocytopoiesis and platelet production through binding to its receptor, Mpl, encoded by the c-mpl proto-oncogene. Circulating levels of TPO are regulated by receptor-mediated uptake and degradation. To better understand this mode of TPO regulat...

journal_title：Blood

authors： Cohen-Solal K,Vitrat N,Titeux M,Vainchenker W,Wendling F

更新日期：1999-05-01 00:00:00

abstract：:We compared the effects of phorbol 12-myristate 13-acetate (PMA) and thrombin with those of nonlytic concentrations of reactive oxygen species (ROS) generated by hypoxanthine (HX)-xanthine oxidase (XO) on the adhesion properties of human umbilical cord vein endothelial cells (HUVEC) to resting polymorphonuclear neutro...

journal_title：Blood

authors： Sellak H,Franzini E,Hakim J,Pasquier C

更新日期：1994-05-01 00:00:00

abstract：:The purpose of this study was to determine the relative merits of idarubicin and daunorubicin in acute myeloid leukemia (AML) therapy. Thirty-two sites provided 214 previously untreated adults with AML aged 15 years or more who were randomized to receive for induction therapy cytarabine 100 mg/m2/d as a continuous 7-d...

journal_title：Blood

authors： Wiernik PH,Banks PL,Case DC Jr,Arlin ZA,Periman PO,Todd MB,Ritch PS,Enck RE,Weitberg AB

更新日期：1992-01-15 00:00:00

abstract：:ADAMTS13 proteolytically regulates the platelet-tethering function of von Willebrand factor (VWF). ADAMTS13 function is dependent upon multiple exosites that specifically bind the unraveled VWF A2 domain and enable proteolysis. We carried out a comprehensive functional analysis of the ADAMTS13 cysteine-rich (Cys-rich)...

journal_title：Blood

authors： de Groot R,Lane DA,Crawley JT

更新日期：2015-03-19 00:00:00

abstract：:A model of Protein C (PC) activation in vivo was used to investigate the complexing of activated PC (APC) with its plasma inhibitors, PC inhibitor (PCI) and alpha 1-antitrypsin (alpha 1AT). Chimpanzees were infused with a bolus of activated factor X (F.Xa) together with vesicles of phosphatidylcholine and phosphatidyl...

journal_title：Blood

authors： Hoogendoorn H,Nesheim ME,Giles AR

更新日期：1990-06-01 00:00:00

abstract：:The peripheral blood of acute myeloblastic leukemia (AML) patients often contains large numbers of two distinct cell populations, both capable of forming colonies in culture under similar conditions. The first population consists of the precursors of blast cells and has specificity for AML; the second population consi...

journal_title：Blood

authors： Minden MD,Buick RN,McCulloch EA

更新日期：1979-07-01 00:00:00

abstract：:Although hereditary spherocytosis (HS) is a common disorder of the red cell membrane, its clinical and biologic expression at birth and in early infancy has received little attention. In order to obtain insights into the natural history of HS during infancy, we studied 46 neonates, 39 from families in which 1 of the p...

journal_title：Blood

authors： Delhommeau F,Cynober T,Schischmanoff PO,Rohrlich P,Delaunay J,Mohandas N,Tchernia G

更新日期：2000-01-15 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：:We have previously shown that the plant-derived compound parthenolide (PTL) can impair the survival and leukemogenic activity of primary human acute myeloid leukemia (AML) stem cells. However, despite the activity of this agent, PTL also induces cellular protective responses that likely function to reduce its overall ...

journal_title：Blood

authors： Hassane DC,Sen S,Minhajuddin M,Rossi RM,Corbett CA,Balys M,Wei L,Crooks PA,Guzman ML,Jordan CT

更新日期：2010-12-23 00:00:00

abstract：:Hairy cell leukemia (HCL) is marked by near 100% mutational frequency of BRAFV600E mutations. Recurrent cooperating genetic events that may contribute to HCL pathogenesis or affect the clinical course of HCL are currently not described. Therefore, we performed whole exome sequencing to explore the mutational landscape...

journal_title：Blood

authors： Dietrich S,Hüllein J,Lee SC,Hutter B,Gonzalez D,Jayne S,Dyer MJ,Oleś M,Else M,Liu X,Słabicki M,Wu B,Troussard X,Dürig J,Andrulis M,Dearden C,von Kalle C,Granzow M,Jauch A,Fröhling S,Huber W,Meggendorfer M,Hafe

更新日期：2015-08-20 00:00:00

abstract：:Recent studies have reported that the pharmacokinetics of high-dose busulfan in bone marrow transplantation (BMT) are age-dependent: with the usual dosage of 16 mg/kg over 4 days, systemic exposure is two to four times lower in children than in adults. Data suggested that the dose of busulfan should rather be calculat...

journal_title：Blood

authors： Vassal G,Deroussent A,Challine D,Hartmann O,Koscielny S,Valteau-Couanet D,Lemerle J,Gouyette A

更新日期：1992-05-01 00:00:00

abstract：:The role of thrombophilia testing in predicting catheter-related deep vein thrombosis (DVT) after an incident (ie, first) catheter-related DVT in children remains unclear. The present study investigated the association between thrombophilia and recurrent catheter-related DVT. Children with thrombophilia testing, perfo...

journal_title：Blood

authors： Avila ML,Amiri N,Stanojevic S,Vu TT,Barron K,Krol P,Yue N,Williams S,Brandão LR

更新日期：2018-06-14 00:00:00

abstract：:Lipid rafts are detergent-resistant, cholesterol- and sphingolipid-rich membrane domains that are involved in important cellular processes such as signal transduction and intracellular trafficking. Stomatin, a major lipid-raft component of erythrocytes and epithelial cells, is also an abundant platelet protein. Micros...

journal_title：Blood

authors： Mairhofer M,Steiner M,Mosgoeller W,Prohaska R,Salzer U

更新日期：2002-08-01 00:00:00

abstract：:Hepatitis B virus (HBV)-infected individuals are occasionally used as donors for bone marrow transplantation (BMT). We studied the rate of HBV infection and the clinical expression of the associated liver disease in patients receiving marrow from HBsAg+ donors. We performed a retrospective survey in 14 BMT units in It...

journal_title：Blood

authors： Locasciulli A,Alberti A,Bandini G,Polchi P,Arcese W,Alessandrino P,Bosi A,Testa M,Bacigalupo A

更新日期：1995-10-15 00:00:00

abstract：:Between February 1986 and March 1990, 56 patients with relapsed Hodgkin's disease treated with high-dose cyclophosphamide, carmustine, and etoposide (CBV) received an autologous peripheral stem cell transplantation (PSCT) rather than an autologous bone marrow transplantation (ABMT) because each patient had a marrow ab...

journal_title：Blood

authors： Kessinger A,Bierman PJ,Vose JM,Armitage JO

更新日期：1991-06-01 00:00:00

abstract：:An as yet single family with a bleeding history is shown to present the characteristic lack of membrane expression of procoagulant phospholipids observed in Scott syndrome. Low prothrombin consumption in the serum of the propositus, a 71-year-old woman, and two of her children was the sole abnormal hemostasis paramete...

journal_title：Blood

authors： Toti F,Satta N,Fressinaud E,Meyer D,Freyssinet JM

更新日期：1996-02-15 00:00:00

abstract：:Naive and memory CD8(+) T cells can undergo programmed activation and expansion in response to a short T-cell receptor stimulus, but the extent to which in vitro programming can qualitatively substitute for an in vivo antigen stimulation remains unknown. We show that self-/tumor-reactive effector memory CD8(+) T cells...

journal_title：Blood

authors： Klebanoff CA,Yu Z,Hwang LN,Palmer DC,Gattinoni L,Restifo NP

更新日期：2009-08-27 00:00:00