Abstract:
:Recently, it was shown that glycoproteins with N-glycans close to the NH(2) terminus can directly enter the calnexin/calreticulin cycle and bypass BiP binding. This should allow efficient secretion of glycoproteins such as factor VIII (FVIII) whose secretion is negatively affected by BiP interaction. Examination of the glycosylation pattern of the NH(2) terminus of FV and FVIII revealed N-glycans at positions 23 and 27 in FV and at position 41 in FVIII. To improve FVIII secretion, a 14-amino-acid-long polypeptide with (G3) or without (G0; control) three N-linked glycosylation consensus sites was inserted upstream of the NH(2) terminus of a B-domain deleted FVIII protein. Expression of G3- and G0-constructs in three different cell lines resulted in the same or even higher expression rate of protein as found for the B-domain deleted FVIII. However, as demonstrated by Western blot analysis, the G3- as well as the G0-protein variants were mainly retained inside the cells in similar amounts. Thus, glycosylation alone does not automatically lead to higher secretion rates, but must be in context to the normal structure of the FVIII protein.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Srour MA,Grupp J,Aburubaiha Z,Albert T,Brondke H,Oldenburg J,Schwaab Rdoi
10.1007/s00277-007-0380-9subject
Has Abstractpub_date
2008-02-01 00:00:00pages
107-12issue
2eissn
0939-5555issn
1432-0584journal_volume
87pub_type
杂志文章abstract::The best treatment option for patients with relapsed or high-grade follicular lymphoma (FL) is unknown. In spite of major advances in the therapy for FL, disease-free survival remains short, and median time to progression is just over a year. Autologous stem cell transplantation in patients with relapsed FL is safe an...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-005-1058-9
更新日期:2005-08-01 00:00:00
abstract::Recently we described a cutaneous T-cell lymphoma expressing the gamma/delta T-cell receptor [5]. The patient suffering from this lymphoma showed low numbers of myeloid and T cells in peripheral blood, while B and NK cells were relatively increased. In vitro culture of the patient's bone marrow (BM) cells revealed a s...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01695808
更新日期:1992-09-01 00:00:00
abstract::Indolent primary cutaneous B cell lymphomas (PCBCL) generally have a good prognosis, but they often relapse leading in some cases to extracutaneous disease and therefore, to poor survival. We developed a prognostic model to improve the therapeutic approach to these lymphomas. Two hundred and seventeen patients with di...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00277-010-1083-1
更新日期:2011-04-01 00:00:00
abstract::MicroRNAs are a class of small non-coding RNAs that are 19-22 nucleotides in length and regulate a variety of biological processes at the post-transcriptional level. MicroRNA dysregulation disrupts normal biological processes, resulting in tumorigenesis. Acute myeloid leukemia is an invasive hematological malignancy c...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-019-03887-5
更新日期:2020-03-01 00:00:00
abstract::We report a factor X (FX)-deficient Chinese family with two novel FX gene (F10) mutations. Two sibling probands had a bleeding tendency since childhood. Both had very low FX:C (<0.01 IU/ml) and FX:Ag (5-6%) levels and were heterozygous for two novel F10 mutations, a 2-bp GC deletion involving nucleotides 33 and 34, le...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0803-1
更新日期:2004-05-01 00:00:00
abstract::Autoantibody against erythrocytes has occasionally been observed in patients with de novo acute myelocytic leukemia (AML). However, it is not clear whether this autoantibody in AML patients induces frank hemolysis (autoimmune hemolytic anemia, AIHA), as seen in lymphoid neoplasms. We present two de novo AML patients w...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF00663016
更新日期:1996-01-01 00:00:00
abstract::Mesenchymal stem cells (MSCs) have emerged as a therapeutic approach in a range of medical fields, including regenerative medicine, cancer, autoimmune diseases, and inflammatory diseases, because of their unique properties of tissue repair and major histocompatibility complex-unmatched immunosuppression. Because both ...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-013-1796-z
更新日期:2013-10-01 00:00:00
abstract::Chronic idiopathic neutropenia of adults (CINA) is a granulocytic disorder characterised by the "unexplained" decrease in the number of circulating neutrophils. Serum inflammatory cytokines and chemokines are increased in CINA. In addition, cytokines gene polymorphisms are associated with increased levels of respectiv...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-005-1066-9
更新日期:2005-10-01 00:00:00
abstract::Several authors have described a particular potential of automated depolarization analysis in detecting malaria infection as part of the routine full blood count (FBC) performed by the Cell-Dyn 4000 analyzer. In these cases, abnormal depolarizing patterns are due to the presence of leukocyte-associated malaria hemozoi...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0786-y
更新日期:2004-05-01 00:00:00
abstract::This paper describes six Indian families in which silent-gene beta-thalassemia was encountered by us in the past 2 years. This stresses the importance of globin-chain synthesis in the diagnosis of beta-thalassemia. Of these six cases, five were diagnosed retrospectively when they had a homozygous beta-thalassemia offs...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01715386
更新日期:1995-01-01 00:00:00
abstract::The impact of intraocular involvement (IOL) in primary CNS lymphoma (PCNSL) has not been sufficiently evaluated. Here, we present the analysis of IOL in the only completed randomized phase III trial in PCNSL. The G-PCNSL-SG1 study evaluated the role of whole-brain radiotherapy in primary therapy of PCNSL. Data of the ...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s00277-014-2212-z
更新日期:2015-03-01 00:00:00
abstract::Although eosinophilia has been reported as a side effect of purine analogues, there is no report on fludarabine-induced eosinophilia in chronic lymphocytic leukemia (CLL). During chemotherapy with fludarabine and cyclophosphamide, we observed two cases of significant eosinophilia. A 67-year-old patient with CLL develo...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s002770050602
更新日期:1999-10-01 00:00:00
abstract::Sickle cell patients are characterized by stress erythropoiesis involving cytokines, growth factors, and adhesion molecules. We set out to determine whether serum soluble vascular cell adhesion molecule-1 (sVCAM-1) levels, which are inversely related to red blood cell counts in sickle cell disease (SCD), reflect eryth...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0610-8
更新日期:2003-03-01 00:00:00
abstract::Primary non-Hodgkin's lymphoma (NHL) of the genital tract is a rare entity. Etiology and pathogenesis of these NHLs are unknown, although there might be a possible association between chronic inflammation and lymphomas. The most common histological subtype is the diffuse large B-cell lymphoma. We report two cases of u...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-009-0741-7
更新日期:2009-12-01 00:00:00
abstract::Bortezomib is one of the most widely used novel drugs for the treatment of multiple myeloma (MM). However, twice-weekly intravenous administration is associated with innegligible adverse events and treatment discontinuation. We therefore evaluated the long-term efficacy and feasibility of reduced frequency treatment w...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00277-016-2661-7
更新日期:2016-05-01 00:00:00
abstract::Lower dosage of total body irradiation (TBI) and chemotherapy in reduced-intensity conditioning (RIC) regimens prior to allogeneic stem cell transplantation have reduced the toxicity of the conditioning and non-relapse mortality. The FLAMSA-RIC protocol for high-risk patients with acute myeloid leukemia (AML) and myel...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-011-1253-9
更新日期:2012-01-01 00:00:00
abstract::Follicular lymphoma (FL) is generally considered an indolent disorder. With modern day treatments, long remissions are often achieved both in the front-line and relapsed setting. However, a subset of patients has a more aggressive course and a worse outcome. Their identification is the main purpose of modern day progn...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-017-3154-z
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:Low folate intake and changes in folate metabolism due to polymorphisms in the methylentetrahydrofolate reductase (MTHFR) gene have been associated with myelomagenesis. However, controversial data have been published regarding a protective role of variant alleles of MTHFR on MM. PATIENTS AND METHODS:To inve...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-006-0097-1
更新日期:2006-07-01 00:00:00
abstract::Because of the widespread use of immunosuppressive drugs, CMV infection is one of the most important causes of morbidity and mortality in patients with haematological malignancies worldwide. The aim of the study was to retrospectively analyse the epidemiology of CMV infection in haematological patients. Between 2008 a...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-016-2831-7
更新日期:2017-01-01 00:00:00
abstract::Established risk factors for thrombosis in essential thrombocythemia (ET) include age (≥ 60 years) and previous vascular events. Recently, also leukocytosis has been proposed in risk stratification of ET patients. We report a retrospective study on 532 ET patients followed for a median of 7.6 years. Sixty-four patient...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-010-1154-3
更新日期:2011-08-01 00:00:00
abstract::Due to their homing properties, extranodal marginal zone B-cell lymphoma (MZBL) of mucosa-associated lymphoid tissue (MALT) type remain localized for long periods of time, and therefore have an excellent prognosis. However, if generalization and/or transformation into a diffuse large-cell lymphoma occurs, the prognosi...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770000203
更新日期:2000-12-01 00:00:00
abstract::Extreme thrombocytosis (ExT) has been associated with an increased bleeding risk in myeloproliferative neoplasm (MPN) patients and is included in the high risk category in treatment guidelines. Treatment of patients with ExT has not been studied in prospective trials. To study physicians' approaches to ExT, we distrib...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-016-2826-4
更新日期:2017-01-01 00:00:00
abstract::The optimal treatment strategy for elderly patients with natural killer/T-cell lymphoma (NKTCL) remains to be established. A total of 63 elderly patients with newly diagnosed NKTCL were retrospectively reviewed. Among the patients with stage I-II disease, 58.3 % received radiotherapy (RT) ± chemotherapy, and 41.7 % re...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-015-2395-y
更新日期:2015-09-01 00:00:00
abstract::In vitro studies suggest that haploinsufficiency is involved in the pathogenesis of myelodysplastic syndromes (MDS). In patients with del5q cytogenetic abnormality, RPS-14 and microRNAs (miRNAs) play a major role. In a multicenter phase II single-arm trial with lenalidomide in anemic primary del5q MDS patients with lo...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-012-1569-0
更新日期:2013-01-01 00:00:00
abstract::FLT3 and its ligand (FL) are one of the regulators of normal hematopoiesis. Ligand-independent activation of FLT3 occurs in about 30% of acute myeloid leukemia cases and is one goal for selectively targeted therapies. However, the function of FLT3/FL in the regulation of non-malignant immature hematopoietic cells is p...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-008-0605-6
更新日期:2009-03-01 00:00:00
abstract::Platelet concentrates were pretreated with a stable synthetic prostacyclin analogue (Iloprost) at two different concentrations before the second centrifugation step (pelleting step) of preparation. This resulted in loss of platelet sensitivity to aggregating agents. To mimic the situation after transfusion and to asse...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01695474
更新日期:1992-06-01 00:00:00
abstract::Diagnosis of thrombotic thrombocytopenic purpura (TTP) is usually based upon the clinical features, and does not always involve histopathological evidence. We recently had experience with a patient who developed the five signs characteristic for TTP. He had been treated for liver cirrhosis associated with chronic hepa...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770100323
更新日期:2001-08-01 00:00:00
abstract::Isocitrate dehydrogenase IDH 1 and IDH 2 mutations were reported in several cancer forms, especially in hematological malignancies, but were never been investigated in familial aggregation. The aim of this study is to determine whether germline isocitrate dehydrogenase genes mutations are involved.We targeted IDH1 and...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-016-2813-9
更新日期:2016-12-01 00:00:00
abstract::Ruxolitinib is a promising option for treating steroid-refractory acute graft-versus-host disease (SR-aGVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). In this study, we describe ruxolitinib treatment for SR-aGVHD in HSCT patients with Epstein-Barr virus-associated hemophagocytic lymphohisti...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-019-03864-y
更新日期:2020-02-01 00:00:00
abstract::Hypermethylation of CpG islands near gene promoter regions is associated with transcriptional inactivation and represents an important mechanism of gene silencing in carcinogenesis. Such epigenetic phenomena can act alongside DNA mutations and deletions to disrupt tumor-suppressor gene function. The methylation status...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-005-0005-0
更新日期:2005-12-01 00:00:00