The Senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients.

Abstract:

:We have previously determined that in African sickle cell anemia (SS) patients three different beta-like globin gene cluster haplotypes are associated with different percent G gamma (one of the two types of non-alpha chains comprising hemoglobin F [HbF]), mean percent HbF, and percent dense cells. We report now that in adult New York SS patients, the presence of at least one chromosome with the Senegal haplotype is associated with higher Hb levels (1.2 g/dL higher) than is found for any other non-Senegal haplotype (P less than .004). The percent reticulocytes and the serum bilirubin levels were lower in these patients. When the effect of alpha-gene number was analyzed by examining a sample of SS patients with concomitant alpha-thalassemia, the same results were obtained. Because the HbF level is significantly higher among the Senegal haplotype carriers in this sample, the inhibitory effect on sickling of this Hb variant may be one of the reasons for the haplotype effect. We conclude that the Senegal beta-like globin gene cluster haplotype is associated with an amelioration of the hemolytic anemia that characterizes sickle cell disease.

journal_name

Blood

journal_title

Blood

authors

Nagel RL,Erlingsson S,Fabry ME,Croizat H,Susuka SM,Lachman H,Sutton M,Driscoll C,Bouhassira E,Billett HH

subject

Has Abstract

pub_date

1991-03-15 00:00:00

pages

1371-5

issue

6

eissn

0006-4971

issn

1528-0020

journal_volume

77

pub_type

杂志文章

相关文献

BLOOD文献大全
  • Tissue type plasminogen activator regulates myeloid-cell dependent neoangiogenesis during tissue regeneration.

    abstract::Ischemia of the heart, brain, and limbs is a leading cause of morbidity and mortality worldwide. Treatment with tissue type plasminogen activator (tPA) can dissolve blood clots and can ameliorate the clinical outcome in ischemic diseases. But the underlying mechanism by which tPA improves ischemic tissue regeneration ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2009-08-236851

    authors: Ohki M,Ohki Y,Ishihara M,Nishida C,Tashiro Y,Akiyama H,Komiyama H,Lund LR,Nitta A,Yamada K,Zhu Z,Ogawa H,Yagita H,Okumura K,Nakauchi H,Werb Z,Heissig B,Hattori K

    更新日期:2010-05-27 00:00:00

  • FGFR2 genotype and risk of radiation-associated breast cancer in Hodgkin lymphoma.

    abstract::Women treated at young ages with supradiaphragmatic radiotherapy for Hodgkin lymphoma (HL) have a highly increased risk of breast cancer. For personalized advice and follow-up regimens for patients, information is needed on how the radiotherapy-related risk is affected by other breast cancer risk factors. Genome-wide ...

    journal_title:Blood

    pub_type: 杂志文章,meta分析

    doi:10.1182/blood-2011-10-383380

    authors: Ma YP,van Leeuwen FE,Cooke R,Broeks A,Enciso-Mora V,Olver B,Lloyd A,Broderick P,Russell NS,Janus C,Ashworth A,Houlston RS,Swerdlow AJ

    更新日期:2012-01-26 00:00:00

  • Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation.

    abstract::Although mycosis fungoides (MF) is typically an indolent disease, patients with advanced-stage disease (stages IIB-IVB), including Sézary syndrome (SS), often have a poor outcome. A 31-year, retrospective analysis of our cutaneous lymphoma database, of 297 patients with MF and SS, was undertaken to study long-term out...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2008-05-154609

    authors: Arulogun SO,Prince HM,Ng J,Lade S,Ryan GF,Blewitt O,McCormack C

    更新日期:2008-10-15 00:00:00

  • Hematopoietic stem cell transplantation does not restore dystrophin expression in Duchenne muscular dystrophy dogs.

    abstract::Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene on the X-chromosome that result in skeletal and cardiac muscle damage and premature death. Studies in mice, including the mdx mouse model of DMD, have demonstrated that circulating bone marrow-derived cells can participate in skeletal musc...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2004-06-2247

    authors: Dell'Agnola C,Wang Z,Storb R,Tapscott SJ,Kuhr CS,Hauschka SD,Lee RS,Sale GE,Zellmer E,Gisburne S,Bogan J,Kornegay JN,Cooper BJ,Gooley TA,Little MT

    更新日期:2004-12-15 00:00:00

  • Interleukin-3 expression by activated T cells involves an inducible, T-cell-specific factor and an octamer binding protein.

    abstract::Interleukin-3 (IL-3) is exclusively expressed by activated T and natural killer cells, a function that is tightly controlled both in a lineage-specific and in a stimulation-dependent manner. We have investigated the protein binding characteristics and functional importance of the ACT-1-activating region of the IL-3 pr...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Davies K,TePas EC,Nathan DG,Mathey-Prevot B

    更新日期:1993-02-15 00:00:00

  • CD4+CD25+ regulatory T-cell deficiency in patients with hepatitis C-mixed cryoglobulinemia vasculitis.

    abstract::Patients who are chronically infected with hepatitis C virus (HCV) often develop mixed cryoglobulinemia (MC), a B-cell proliferative disorder with polyclonal activation and autoantibody production. We investigated if MC is associated with a deficit of CD4(+)CD25(+) immunoregulatory T (Treg) cells, which have been show...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2003-07-2598

    authors: Boyer O,Saadoun D,Abriol J,Dodille M,Piette JC,Cacoub P,Klatzmann D

    更新日期:2004-05-01 00:00:00

  • Tissue factor pathway inhibitor dose-dependently inhibits coagulation activation without influencing the fibrinolytic and cytokine response during human endotoxemia.

    abstract::Inhibition of the tissue factor pathway has been shown to attenuate the activation of coagulation and to prevent death in a gram-negative bacteremia primate model of sepsis. It has been suggested that tissue factor influences inflammatory cascades other than the coagulation system. The authors sought to determine the ...

    journal_title:Blood

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:

    authors: de Jonge E,Dekkers PE,Creasey AA,Hack CE,Paulson SK,Karim A,Kesecioglu J,Levi M,van Deventer SJ,van Der Poll T

    更新日期:2000-02-15 00:00:00

  • Use of a sensitive bioimmunoabsorbent assay to isolate and characterize monoclonal antibodies to biologically active human erythropoietin.

    abstract::At present, one of the most sensitive assays for human erythropoietin (Ep) is a bioassay that measures the Ep-dependent proliferation of spleen cells from phenylhydrazine-treated mice after 24 hours in culture. We describe how this assay can be used as the basis of a very sensitive method for detecting mouse antibodie...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Wognum AW,Lansdorp PM,Eaves CJ,Krystal G

    更新日期:1988-06-01 00:00:00

  • Ligand-induced internalization selects use of common receptor neuropilin-1 by VEGF165 and semaphorin3A.

    abstract::Neuropilin-1 (Npn-1) is a receptor shared by class 3 semaphorins and heparin-binding forms of vascular endothelial growth factor (VEGF), protein families that regulate endothelial and neuronal-cell function. Ligand interaction with Npn-1 dictates the choice of signal transducer; plexins transduce semaphorin signals, a...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2005-10-4113

    authors: Narazaki M,Tosato G

    更新日期:2006-05-15 00:00:00

  • Necdin, a p53 target gene, regulates the quiescence and response to genotoxic stress of hematopoietic stem/progenitor cells.

    abstract::We recently defined a critical role for p53 in regulating the quiescence of adult hematopoietic stem cells (HSCs) and identified necdin as a candidate p53 target gene. Necdin is a growth-suppressing protein and the gene encoding it is one of several that are deleted in patients with Prader-Willi syndrome. To define th...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2011-11-393983

    authors: Asai T,Liu Y,Di Giandomenico S,Bae N,Ndiaye-Lobry D,Deblasio A,Menendez S,Antipin Y,Reva B,Wevrick R,Nimer SD

    更新日期:2012-08-23 00:00:00

  • Variable sensitivity of FLT3 activation loop mutations to the small molecule tyrosine kinase inhibitor MLN518.

    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

    pub_type: 杂志文章

    doi:10.1182/blood-2003-12-4446

    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

  • Mig, the monokine induced by interferon-gamma, promotes tumor necrosis in vivo.

    abstract::Mig, the monokine induced by interferon-gamma, is a CXC chemokine active as a chemoattractant for activated T cells. Mig is related functionally to interferon-inducible protein 10 (IP-10), with which it shares a receptor, CXCR3. Previously, IP-10 was found to have antitumor activity in vivo. In the present study, muri...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Sgadari C,Farber JM,Angiolillo AL,Liao F,Teruya-Feldstein J,Burd PR,Yao L,Gupta G,Kanegane C,Tosato G

    更新日期:1997-04-15 00:00:00

  • Tissue-type plasminogen activator (t-PA) induces stromelysin-1 (MMP-3) in endothelial cells through activation of lipoprotein receptor-related protein.

    abstract::Tissue-type plasminogen activator (t-PA) is approved for treatment of ischemic stroke patients, but it increases the risk of intracranial bleeding (ICB). Previously, we have shown in a mouse stroke model that stromelysin-1 (matrix metalloproteinase-3 [MMP-3]) induced in endothelial cells was critical for ICB induced b...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2009-02-203919

    authors: Suzuki Y,Nagai N,Yamakawa K,Kawakami J,Lijnen HR,Umemura K

    更新日期:2009-10-08 00:00:00

  • Identification of the molecular genetic defect of patients with methemoglobin M-Kankakee (M-Iwate), alpha87 (F8) His --> Tyr: evidence for an electrostatic model of alphaM hemoglobin assembly.

    abstract::We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, alpha87 (F8) His --> Tyr] resides in the alpha1-globin gene. The proportion of Hb M observed is higher than that predicted for an alpha1-globin variant. Our evidence suggests that the greater-than-expected proportion of ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Ameri A,Fairbanks VF,Yanik GA,Mahdi F,Thibodeau SN,McCormick DJ,Boxer LA,McDonagh KT

    更新日期:1999-09-01 00:00:00

  • Prevalence of heterozygotes for hemochromatosis in the white population of the United States.

    abstract::In previous studies, the prevalence of HLA-linked hemochromatosis, thought to be the most common genetic illness in whites, has been estimated by identifying homozygotes in the population. Because not all homozygotes express the disease phenotypically, the accuracy of these estimates is uncertain. We analyzed the dist...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: McLaren CE,Gordeuk VR,Looker AC,Hasselblad V,Edwards CQ,Griffen LM,Kushner JP,Brittenham GM

    更新日期:1995-09-01 00:00:00

  • Bortezomib induces canonical nuclear factor-kappaB activation in multiple myeloma cells.

    abstract::Bortezomib is a proteasome inhibitor with remarkable preclinical and clinical antitumor activity in multiple myeloma (MM) patients. The initial rationale for its use in MM was inhibition of nuclear factor (NF)-kappaB activity by blocking proteasomal degradation of inhibitor of kappaBalpha (IkappaBalpha). Bortezomib in...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2009-01-199604

    authors: Hideshima T,Ikeda H,Chauhan D,Okawa Y,Raje N,Podar K,Mitsiades C,Munshi NC,Richardson PG,Carrasco RD,Anderson KC

    更新日期:2009-07-30 00:00:00

  • Inhibition of immature erythroid progenitor cell proliferation by macrophage inflammatory protein-1alpha by interacting mainly with a C-C chemokine receptor, CCR1.

    abstract::Several lines of evidence indicate that macrophage inflammatory protein-1alpha (MIP-1alpha) modulates the proliferation of hematopoietic progenitor cells, depending on their maturational stages. To clarify the mechanisms for the modulation of hematopoiesis by this chemokine, we examined the expression of a receptor fo...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Su S,Mukaida N,Wang J,Zhang Y,Takami A,Nakao S,Matsushima K

    更新日期:1997-07-15 00:00:00

  • Polymorphisms in the chemokine (C-X-C motif) ligand 10 are associated with invasive aspergillosis after allogeneic stem-cell transplantation and influence CXCL10 expression in monocyte-derived dendritic cells.

    abstract::Patients after allogeneic stem-cell transplantation (alloSCT) have an increased risk for invasive aspergillosis (IA). Here, recipients of an allograft with IA (n=81) or without IA (n=58) were screened for 84 single nucleotide polymorphisms in 18 immune relevant genes. We found 3 markers in chemokine (C-X-C motif) liga...

    journal_title:Blood

    pub_type: 杂志文章,多中心研究

    doi:10.1182/blood-2007-05-090928

    authors: Mezger M,Steffens M,Beyer M,Manger C,Eberle J,Toliat MR,Wienker TF,Ljungman P,Hebart H,Dornbusch HJ,Einsele H,Loeffler J

    更新日期:2008-01-15 00:00:00

  • Development of a lethal mast cell disease in mice reconstituted with bone marrow cells expressing the v-erbB oncogene.

    abstract::An animal model for malignant mastocytosis is described in mice reconstituted with bone marrow cells expressing the v-erbB oncogene. The lethal mast cell disease is characterized by massive infiltration of bone marrow, spleen, and several other visceral organs by connective tissue mast cells, which normally reside in ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: von Rüden T,Kandels S,Radaszkiewicz T,Ullrich A,Wagner EF

    更新日期:1992-06-15 00:00:00

  • Drug-induced thrombocytopenia: development of a novel NOD/SCID mouse model to evaluate clearance of circulating platelets by drug-dependent antibodies and the efficacy of IVIG.

    abstract::Drug-induced immune thrombocytopenia (DITP) is an adverse drug effect mediated by drug-dependent antibodies. Intravenous immunoglobulin (IVIG) is frequently used to treat DITP and primary immune thrombocytopenia (ITP). Despite IVIG's proven beneficial effects in ITP, its efficacy in DITP is unclear. We have establishe...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2010-02-268326

    authors: Liang SX,Pinkevych M,Khachigian LM,Parish CR,Davenport MP,Chong BH

    更新日期:2010-09-16 00:00:00

  • Divergent effects of interleukin-4 (IL-4) on the granulocyte colony-stimulating factor and IL-3-supported myeloid colony formation from normal and leukemic bone marrow cells.

    abstract::Human recombinant interleukin-4 (IL-4) was studied for its effects on myeloid progenitor cells from normal and leukemic bone marrow cells in the presence and absence of additional growth factors. IL-4 itself did not support myeloid cluster or colony formation (CFU-GM). However, cultures supplied with IL-4 (300 U/mL) a...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Vellenga E,de Wolf JT,Beentjes JA,Esselink MT,Smit JW,Halie MR

    更新日期:1990-02-01 00:00:00

  • Myeloma bone disease and proteasome inhibition therapies.

    abstract::Bone disease is one of the most debilitating manifestations of multiple myeloma. A complex interdependence exists between myeloma bone disease and tumor growth, creating a vicious circle of extensive bone destruction and myeloma progression. Proteasome inhibitors have recently been shown to promote bone formation in v...

    journal_title:Blood

    pub_type: 杂志文章,评审

    doi:10.1182/blood-2007-03-067710

    authors: Terpos E,Sezer O,Croucher P,Dimopoulos MA

    更新日期:2007-08-15 00:00:00

  • Sequestration of p27Kip1 protein by cyclin D1 in typical and blastic variants of mantle cell lymphoma (MCL): implications for pathogenesis.

    abstract::p27 is a cyclin-dependent kinase inhibitor that plays a critical role in regulating G(1)/S progression, and whose activity is, in part, regulated through interactions with D-type cyclins. Mantle cell lymphoma (MCL) is characterized by the t(11;14) translocation resulting in deregulated cyclin D1. We previously showed ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2002-01-0263

    authors: Quintanilla-Martinez L,Davies-Hill T,Fend F,Calzada-Wack J,Sorbara L,Campo E,Jaffe ES,Raffeld M

    更新日期:2003-04-15 00:00:00

  • Identification of human chronic myelogenous leukemia progenitor cells with hemangioblastic characteristics.

    abstract::Overwhelming evidence from leukemia research has shown that the clonal population of neoplastic cells exhibits marked heterogeneity with respect to proliferation and differentiation. There are rare stem cells within the leukemic population that possess extensive proliferation and self-renewal capacity not found in the...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2004-07-2514

    authors: Fang B,Zheng C,Liao L,Han Q,Sun Z,Jiang X,Zhao RC

    更新日期:2005-04-01 00:00:00

  • Shared idiotype expression by chronic lymphocytic leukemia and B-cell lymphoma.

    abstract::Antiidiotype (Id) antibodies identify unique determinants within the surface immunoglobulin (Ig) that are present on B-cell tumors. Anti-Ids have been used for diagnosis and therapy of B-cell lymphoma and leukemia. A panel of 29 anti-Id monoclonal antibodies (MoAbs) that recognize shared idiotypes (SIds) on B-cell lym...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Chatterjee M,Barcos M,Han T,Liu XL,Bernstein Z,Foon KA

    更新日期:1990-11-01 00:00:00

  • Effects of neuraminidase on the regulation of erythropoiesis.

    abstract::In this article, we present evidence that sialic acid-containing surface components play a role in the regulation of erythropoiesis. A 1-hr exposure of mouse bone marrow cells to high concentrations of neuraminidase reduced erythroid colony formation. Coculture of 10(6) untreated thymocytes with neuraminidase-treated ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: LaRussa VF,Sieber F,Sensenbrenner LL,Sharkis SJ

    更新日期:1984-04-01 00:00:00

  • Thrombin stimulation of p38 MAP kinase in human platelets is mediated by ADP and thromboxane A2 and inhibited by cGMP/cGMP-dependent protein kinase.

    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

    pub_type: 杂志文章

    doi:10.1182/blood-2006-07-038158

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

    更新日期:2007-01-15 00:00:00

  • Hematopoietic progenitor kinase 1 (HPK1) is required for LFA-1-mediated neutrophil recruitment during the acute inflammatory response.

    abstract::Recruitment of polymorphonuclear neutrophils (PMNs) to sites of acute inflammation critically depends on β2 integrins (CD11/CD18). Recently, the mammalian actin-binding protein 1 (mAbp1) was identified as an important adaptor protein regulating PMN trafficking downstream of β2 integrins. Here, we show that mAbp1 const...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2012-08-451385

    authors: Jakob SM,Pick R,Brechtefeld D,Nussbaum C,Kiefer F,Sperandio M,Walzog B

    更新日期:2013-05-16 00:00:00

  • Rituximab for the treatment of refractory autoimmune hemolytic anemia in children.

    abstract::Autoimmune hemolytic anemia (AIHA) in children is sometimes characterized by a severe course, requiring prolonged administration of immunosuppressive therapy. Rituximab is able to cause selective in vivo destruction of B lymphocytes, with abrogation of antibody production. In a prospective study, we have evaluated the...

    journal_title:Blood

    pub_type: 临床试验,杂志文章

    doi:10.1182/blood-2002-11-3547

    authors: Zecca M,Nobili B,Ramenghi U,Perrotta S,Amendola G,Rosito P,Jankovic M,Pierani P,De Stefano P,Bonora MR,Locatelli F

    更新日期:2003-05-15 00:00:00

  • Detection of minimal residual disease in acute lymphoblastic leukemia using immunoglobulin hypervariable region specific oligonucleotide probes.

    abstract::To develop a sensitive and specific assay for minimal residual disease in acute lymphoblastic leukemia (ALL), we exploited the enormous diversity of genomic sequences created by immune receptor gene rearrangements. To isolate clone-specific sequences, we first synthesized oligonucleotides that match conserved variable...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Jonsson OG,Kitchens RL,Scott FC,Smith RG

    更新日期:1990-11-15 00:00:00