Abstract:
:Human beta-globin was produced in Escherichia coli as a cleavable fusion protein using the expression vector pLcII [Nagai and Thøgersen, Nature 301, p. 810, 1984]. The fusion protein CIIFX beta-globin was purified under denaturing conditions to homogeneity and the authentic beta-globin was liberated by blood coagulation factor Xa. beta-Globin was then folded and reconstituted with haem and alpha-subunits to form fully functional alpha 2 beta 2-tetramers [Nagai et al., Proc. natn. Acad. Sci. USA 82, p. 7252, 1985]. This has enabled us to produce mutants with amino acid substitutions in the beta-subunit at will and in sufficient quantities to study their oxygen-binding properties and three-dimensional structures. We have crystallised three mutants, Hb Nympheas [Cys-93 beta----Ser], Hb Daphne [Cys-93 beta----Ser, His-143 beta----Arg] and Hb Sandra [Cys-93 beta----Ser, Asp-94 beta----Glu], and have solved their structures to high resolution by x-ray crystallography.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Luisi BF,Nagai K,Perutz MFdoi
10.1159/000205851subject
Has Abstractpub_date
1987-01-01 00:00:00pages
85-9issue
2-3eissn
0001-5792issn
1421-9662journal_volume
78pub_type
杂志文章abstract::We have used the gel retardation assay to investigate the binding of nuclear proteins to the duplicated CACCC boxes in the beta-globin gene promoter region. The effect of beta-thalassemia mutations affecting both of these consensus sequences (the -88 C-->T and -101 C-->T mutations) were studied by using appropriate mu...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204237
更新日期:1994-01-01 00:00:00
abstract::The detection of cellular antigens in bone marrow sections depends on the method of embedding, the nature of antigen and antibody, antigen retrieval techniques and the sensitivity of the immunohistochemical method. This study evaluated a fluorescyl-tyramide-enhanced immunostaining method on methyl-methacrylate-embedde...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000097458
更新日期:2007-01-01 00:00:00
abstract::We describe a patient with recurrence of Hodgkin's disease and severe liver disease of unknown origin in whom autoimmune neutropenia developed. Because of possible seronegative viral hepatitis he was treated with high-dose intravenous immunoglobulin instead of steroids. He responded with a prompt but transitory increa...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203968
更新日期:1995-01-01 00:00:00
abstract::The concentration of plasma vitamin B12 bound to transcobalamin II was measured by two methods: QUSO and Heparin Sepharose (HS). The new HS procedure provided a specific, direct measurement of vitamin B12 bound to both R binder and transcobalamin II in plasma. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204522
更新日期:1993-01-01 00:00:00
abstract::Afibrinogenaemia in a female Saudi child is reported. Two siblings died of bleeding. The parents, who are first cousins, a brother and two sisters are asymptomatic heterozygotes with fibrinogen levels of less than 1.5 g/l. The presentation was described and compared to other cases reported. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206624
更新日期:1984-01-01 00:00:00
abstract::Phagocytic activity of leukaemic blasts in 20 adults and 16 children suffering from acute leukaemia was studied in vitro by the use of ferrioxidsaccharate. Most frequently phagocytosing blasts were encountered in myelomonocytic leukaemias. The morphological character of positive blasts resembled often that of monocyto...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208150
更新日期:1975-01-01 00:00:00
abstract::Preliminary results from the first 21 patients of a group of 30 with International Federation of Gynaecology and Obstetrics (FIGO) stage II-IV epithelial ovarian carcinoma and anaemia are reported. Patients entered this open-label, comparative-group, out-patient study and were randomized to receive conventional suppor...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000204782
更新日期:1992-01-01 00:00:00
abstract::Malaria parasites growing inside human erythrocytes differ from mammalian cells in their mode of acquisition of bioavailable iron and in their susceptibility to the antiproliferative action of iron chelators. We have assessed here three major properties associated with these phenomena: (a) the stage-dependent nature o...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000203952
更新日期:1996-01-01 00:00:00
abstract::The effect of 3 different detergents (Triton X-100, Lubrol WX and Nonidet) on brain thromboplastin was examined and compared with the solubilisation due to deoxycholate. The effect of deoxycholate is superior over that of the other examined detergents. Deoxycholate extracts thromboplastic activity from brain tissue to...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208068
更新日期:1975-01-01 00:00:00
abstract::Alpha-thalassemia is very common in the Kuwaiti population, but its influence on anemia of pregnancy has not been previously investigated. We have screened a group of 59 anemic (Hb < 11 g/dl) pregnant women for the alpha-thal-2 (-alpha-3.7 kb) deletion which is the commonest alpha-thal allele in this community, using ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000039757
更新日期:2000-01-01 00:00:00
abstract::Fibroblast-like cells were grown from the bone marrow of acute lymphoblastic leukemia (ALL) patients before, during and off therapy. A diminished growth capacity was observed in cells from patients before and during therapy. In the three groups studied, differences were also observed in the effect of hydrocortisone on...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206284
更新日期:1985-01-01 00:00:00
abstract::Recently, concerns have been raised regarding the potential impairment of neonatal platelet function and the potential risk of bleeding in neonates born to mothers treated with selective serotonin reuptake inhibitors (SSRI). Our aim was to test whether the platelet function of neonates born to SSRI-treated mothers was...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000090929
更新日期:2006-01-01 00:00:00
abstract::Serum IgG, IgM and IgA were determined in 25 patients with homozygous beta thalassemia and 7 with the trait. The levels were increased in homozygous patients and increased further after splenectomy. Serum opsonic activity against Salmonella typhi and staphylococci was impaired in homozygous patients. Splenectomy cause...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206875
更新日期:1983-01-01 00:00:00
abstract::The short-term effect of a domestically produced equine antithymocyte globulin (ATG) was analyzed in 6 patients with acquired severe aplastic anemia (AA). All patients received 5 doses of ATG every other day in a 60-min intravenous infusion. Five peripheral blood immunoregulatory mononuclear cell (MNC) subsets, define...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000205557
更新日期:1989-01-01 00:00:00
abstract::A 69-year-old man with a rare case of lymphoma-type alpha-chain disease was admitted to the hospital with marked cervical and inguinal lymph node swelling. Lymph node biopsy showed marked infiltration of plasma cells, plasmacytoid cells and immunoblastoid cells, alone or in combination. Immunoelectrophoresis and immun...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204814
更新日期:1991-01-01 00:00:00
abstract::Eleven patients with acute myeloid leukaemia (AML) in first complete remission (CR) were treated with alfa-2a-interferon (for short 'interferon') maintenance therapy, at a dose of 3 MU twice to thrice weekly subcutaneously. Adjustments were made to maintain neutrophil counts > 1 x 10(9)/l and platelet counts > 100 x 1...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204473
更新日期:1993-01-01 00:00:00
abstract::In evaluating hypoimmunoglobulinemia in a patient with nonsecretory myeloma, studies of in vitro immunoglobulin synthesis were performed during a 3-year period. Impaired polyclonal response to mitogen stimulation associated with excessive suppressor activity was demonstrable at diagnosis. Despite successful chemothera...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205622
更新日期:1988-01-01 00:00:00
abstract::Iron overload due to regular transfusions of packed red cells can cause multiple organ damage. Iron chelation therapy (ICT) is important in patients with aplastic anemia (AA) who require blood transfusions as supportive management. With the introduction of the oral iron chelator deferasirox, ICT has become more widely...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000342772
更新日期:2013-01-01 00:00:00
abstract::A 69-year-old Japanese female was admitted to our hospital due to a 2-month history of vomiting after eating. Examination of the small intestinal tract revealed a tumor with calcification in the inner portion, from the horizontal portion to the ascending portion of the duodenum, and jejunojejunostomy was performed. Th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000041012
更新日期:1999-01-01 00:00:00
abstract::Increased levels of hemoglobin A(2) (HbA(2)) are present in most beta-thalassemia carriers. The mechanism of this effect is not understood, although the increase may result from transcriptional and posttranscriptional changes. In the present study, we quantitate delta-globin mRNA levels in peripheral-blood-enriched re...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040959
更新日期:1999-01-01 00:00:00
abstract::The management of severe late-onset hemorrhagic cystitis (LO-HC) after allogeneic hematopoietic stem cell transplantation (HSCT) is still challenging. Because mesenchymal stromal cells (MSCs) possess anti-inflammatory and tissue repair-promoting properties, we retrospectively analyzed the efficacy and safety of MSC in...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000362530
更新日期:2015-01-01 00:00:00
abstract::Janus kinases are critical components of signaling pathways that regulate hematopoiesis. Mutations of the non-receptor tyrosine kinase JAK2 are found in many BCR-ABL-negative myeloproliferative neoplasms. Preclinical results support that JAK2 inhibitors could show efficacy in treating chronic myeloproliferative neopla...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000356784
更新日期:2014-01-01 00:00:00
abstract::Avascular necrosis (AVN) of the hip is a common cause of morbidity in sickle cell disease (SCD). Its prevalence increases with age and predisposing factors include coexistent alpha-thalassemia trait, frequent vaso-occlusive crisis and a high hematocrit (Hct). SCD is relatively mild among Kuwaiti patients because of th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000046529
更新日期:2001-01-01 00:00:00
abstract::Two subjects, not previously reported in detail, had severe inherited deficiencies of erythrocyte pyrimidine nucleotidase. This was manifested hematologically by moderate hemolytic anemia with splenomegaly, morphologically by punctate basophilic stippling of Wright's stained erythrocytes, and biochemically by intraery...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207413
更新日期:1980-01-01 00:00:00
abstract::Recent molecular biological investigations have shown that genetic information on the short arm of chromosome 17 is frequently deleted or inactivated in acute myeloid leukaemias and a number of solid tumours. In earlier studies we have found clonal occurrence of a 17p deletion (17p-) in patients with refractory anaemi...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205033
更新日期:1990-01-01 00:00:00
abstract::While sickle cell disease (SCD) is generally mild in most Kuwaitis, because of their elevated fetal Hb levels, avascular necrosis of the femoral head (AVNFH) appears to be a common complication. It was recently documented in 26.7% of Kuwaiti children with SCD. There have, however, been no previous studies of adult pat...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000072406
更新日期:2003-01-01 00:00:00
abstract::All-trans-retinoic acid (ATRA) combined with anthracyclines is currently the standard treatment for acute promyelocytic leukemia (APL). In elderly patients the presence of comorbidities, such as cardiomyopathy or different organ failures, often represents an absolute contraindication to standard chemotherapy. In this ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000097880
更新日期:2007-01-01 00:00:00
abstract::We report a patient with myelodysplastic syndrome (MDS), refractory anaemia with excess blasts in transformation, in whom complete remission (CR) was achieved with the administration of granulocyte colony-stimulating factor (G-CSF). The 76-year-old patient was admitted to our hospital with a fever and a productive cou...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000057637
更新日期:2002-01-01 00:00:00
abstract::High-dose gammaglobulin therapy for patients with idiopathic thrombocytopenic purpura (ITP), introduced by Imbach et al., was applied to 5 adults with chronic refractory ITP to investigate the mechanism of the increase in platelet counts. In 4 of the 5 cases, transient increase in platelet count was observed. Platelet...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206927
更新日期:1983-01-01 00:00:00
abstract::von Willebrand disease (VWD) type 2M is a distinct entity and clearly differs from type 1. The genotype-phenotype correlation for cases with ristocetin cofactor activity (RCo)/antigen (Ag) ratios <0.60 is clear, whereas the von Willebrand factor (VWF):collagen binding (CB)/VWF:Ag ratio is normal in VWD 2M. Typical lab...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000214854
更新日期:2009-01-01 00:00:00