Abstract:
:Biosynthesis of the adhesive glycoprotein von Willebrand factor (vWf) by endothelial cells results in constitutive secretion of small multimers and storage of the largest multimers in rod-shaped granules called Weibel-Palade bodies. This pattern is reproduced by expression of pro-vWf in heterologous cells with a regulated pathway of secretion, that store the recombinant protein in similar elongated granules. In these cells, deletion of the vWf prosequence prevents vWf storage. The prosequence, composed of two homologous domains (D1 and D2), actively participates in vWf multimer formation as well. We expressed deletion mutants lacking either the D1 domain (D2vWf) or the D2 domain (D1vWf) in various cell lines to analyze the relative importance of each domain in vWf multimerization and storage. Both proteins were secreted efficiently without being retained in the endoplasmic reticulum. Despite this, neither multimerized past the dimer stage and they were not stored. We conclude that several segments of the prosequence are jointly involved in vWf multimerization and storage.
journal_name
Thromb Haemostjournal_title
Thrombosis and haemostasisauthors
Journet AM,Saffaripour S,Wagner DDsubject
Has Abstractpub_date
1993-12-20 00:00:00pages
1053-7issue
6eissn
0340-6245issn
2567-689Xjournal_volume
70pub_type
杂志文章abstract::Heparin is one of the oldest biological medicines, and has an established place in the prevention and treatment of venous thrombosis. Low-molecular-weight heparins (LMWH) have been developed by several manufacturers and have advantages in terms of pharmacokinetics and convenience of administration. They have been show...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:10.1160/TH08-01-0032
更新日期:2008-05-01 00:00:00
abstract::The increased risk for deep vein thrombosis (DVT) after orthopaedic surgery has been well documented as well as hypercoagulable state during both total hip arthroplasty (THA) and total knee replacement (TKR). To investigate the influence of the surgical procedure [posterolateral (PL) or lateral (L) approach for THA, u...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1999-06-01 00:00:00
abstract::Heparin-induced thrombocytopenia (HIT), a relatively common complication of heparin therapy, results of platelet activation, via the receptor for the Fc domain of IgG (FcgammaRIIa), by heparin-dependent-antibodies, commonly directed against the heparin-platelet factor 4 (H-PF4) antigenic complex. Our strategy was to u...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:2001-06-01 00:00:00
abstract::Endotoxin (LPS) and interleukin-1 beta (IL-1 beta) increased in a dose-dependent manner the expression of tissue factor, an ubiquitous membrane-anchored glycoprotein that initiates blood coagulation at the surface of human umbilical vein endothelial cells (HUVEC and human peripheral blood mononuclear cells (PBMC). Ech...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1996-02-01 00:00:00
abstract::Although sepsis-induced release of nitric oxide (NO) is known to have an antithrombotic effect, it is unknown if NO exerts this same effect under physiological conditions. We have there-fore attempted to determine whether or not NO protects against thrombus formation in normal Wistar rats injected with various amounts...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH03-05-0292
更新日期:2004-01-01 00:00:00
abstract::Heparin and its derivatives have been shown to inhibit angiogenesis and metastasis formation. Accordingly, we investigated the effect of heparin fragments containing 4 to 22 monomers on human melanoma cell proliferation, migration and invasion in vitro as well as on the in vivo metastatic potential in a SCID mouse mod...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH09-01-0059
更新日期:2009-12-01 00:00:00
abstract::The Pulmonary Embolism Severity Index (PESI) is a validated clinical prognostic model for patients with acute pulmonary embolism (PE). Our goal was to assess the PESI's inter-rater reliability in patients diagnosed with PE. We prospectively identified consecutive patients diagnosed with PE in the emergency department ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH10-07-0426
更新日期:2010-12-01 00:00:00
abstract::Atherosclerotic plaques are found at distinct locations in the arterial system, despite the exposure to systemic risk factors of the entire vascular tree. From the study of arterial bifurcation regions, emerges ample evidence that haemodynamics are involved in the local onset and progression of the atherosclerotic dis...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:10.1160/TH15-07-0597
更新日期:2016-03-01 00:00:00
abstract::We tested different types of clot for their ability to provoke procoagulant activity in platelets: normal clots from platelet poor plasma (des AABB- or fibrin II clots), similar clots in which the adsorbed thrombin has been inhibited by hirudin, and clots obtained by the action of two snake venom enzymes that release ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1995-09-01 00:00:00
abstract::Lipid oxidation products and in particular oxidized phospholipids (OxPL) are increasingly recognized as inducers of chronic inflammation characteristic of atherosclerosis. OxPL stimulate production of chemokines and adhesion of monocytes to endothelial cells. However, accumulating data suggest that, in addition to the...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:
更新日期:2007-03-01 00:00:00
abstract::A congenitally abnormal fibrinogen was isolated from blood of a young man with deep-vein thrombosis. Two other affected members of his family had three episodes of severe arterial thrombosis. The fibrinogen showed a delayed clotting by thrombin, but a normal clotting by Arvin, Reptilase, and prothrombin-staphylocoagul...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1986-02-28 00:00:00
abstract::Recombinant factor VIIa (rVIIa) has been reported to be clinically effective and safe in haemophilic patients with inhibitor antibodies. Compared to activated prothrombin complex concentrates the risk of thrombotic complications seems to be very low after rVIIa administration. Determination of free thrombin generation...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1999-02-01 00:00:00
abstract:OBJECTIVE: Blood monocyte subsets are emerging as biomarkers of cardiovascular inflammation. However, our understanding of human monocyte heterogeneity and their immunophenotypic features under healthy and inflammatory conditions is still evolving. RATIONALE: In this study, we sought to investigate the immunophenome o...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1055/s-0039-1700871
更新日期:2020-01-01 00:00:00
abstract::Our initial finding that CD40- and CD40 ligand (CD40L)-deficient mice displayed prolonged tail bleeding and platelet function analyzer (PFA-100) closure times prompted us to further investigate the role of the CD40-CD40L dyad in primary hemostasis and platelet function. Recombinant human soluble CD40L (rhsCD40L), chem...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH04-12-0774
更新日期:2005-06-01 00:00:00
abstract::The blockade of platelet membrane glycoprotein IIb/IIIa by a monoclonal antibody, 7E3, was measured by flow cytometry using a fluorescein isothiocyanate-conjugated disintegrin, FITC-crotavirin, as the probe. After treatment of platelets with 7E3 or 7E3 F(ab')2, there is a good correlation between the inhibition of pla...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1996-10-01 00:00:00
abstract::We investigated the prevalence of the new recently reported mutation in the prothrombin gene (20210 A) in a sample of 116 unrelated patients with venous thromboembolism. We found 20 heterozygous carriers (17.2%, CI 95% 10.4-21.1). In comparison, we observed 13 carriers among 201 healthy unmatched controls (6.5%, CI 3....
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1998-09-01 00:00:00
abstract::Congenital afibrinogenemia and severe hypofibrinogenemia are severe bleeding disorders characterized by either undetectable or very low levels of fibrinogen in patients' plasma and platelets. A majority of the reported cases are caused by mutations in the fibrinogen Aalpha chain. In this study, we identified a genetic...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH06-01-0020
更新日期:2006-06-01 00:00:00
abstract::Washed human platelets have been labeled with either 32Pi or glycerol-1-14C and the distribution of the label in the phospholipids determined. 32Pi was introduced primarily into polyphosphoinositides, i.e. di- and triphosphoinositide, whereas the label from glycerol which indicates de novo synthesis of lipid molecules...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1976-04-30 00:00:00
abstract::In the last decade, the endothelial cell protein C/activated protein C receptor (EPCR) has received considerable attention. The role initially attributed to EPCR, i.e. the enhancement of protein C (PC) activation by the thrombin-thrombomodulin complex on the surface of the large vessels, although important, did not go...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:10.1160/TH11-11-0766
更新日期:2012-05-01 00:00:00
abstract::Viper venoms contain a variety of platelet binding proteins including those which bind to platelet GPIb/GPIX. Most of these proteins inhibit von Willebrand factor mediated platelet agglutination. Here we report the primary structures of unique members of this family, alboaggregins A and B, isolated from Trimeresurus a...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1998-03-01 00:00:00
abstract::A factor VIII inhibitor has been found in a patient with an unusual combination of factor VIII-related properties. The inhibitor is directed specifically against the clotting activity (VIII:C) of the factor VIII complex. It behaves in a similar fashion to high responding inhibitors of factor VIII seen in haemophilia A...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1986-04-30 00:00:00
abstract::Heparin cofactor II (HC II) is a heparin-dependent inhibitor of thrombin, distinct from antithrombin III (AT III). This study was designed to evaluate its metabolism in healthy subjects. Purified HC II was labelled with 125I by the lactoperoxidase-glucose oxidase technique. The biological activity of the HC II was unc...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1985-10-30 00:00:00
abstract::Human fibrinogen was subjected to limited proteolytic attack by thrombin, batroxobin or Agkistrodon contortrix thrombin-like enzyme, yielding desAB-, desA- or desB-fibrin monomers, respectively. Turbidity curves demonstrated that, with all three enzymes, the polymerization process was strongly accelerated by increasin...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1982-04-30 00:00:00
abstract::Catheter-directed thrombolysis has gained increasing acceptance for the treatment of patients who present with vascular occlusion; however, intravenous injection may be preferable in selected patients. Recombinant prourokinase (r-proUK) is a recently-developed fibrin-selective thrombolytic agent with specificity for c...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1999-02-01 00:00:00
abstract::Carboxypeptidase U (CPU, CPB2, TAFIa) is a basic carboxypeptidase that is able to attenuate fibrinolysis. The inactive precursor procarboxypeptidase U is converted to its active form by thrombin, the thrombin-thrombomodulin complex or plasmin. The aim of this study was to investigate and characterise the time course o...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH17-02-0097
更新日期:2017-07-26 00:00:00
abstract:BACKGROUND:Paravalvular aortic regurgitation (PVAR) remains a frequent postprocedural concern following transcatheter aortic valve replacement (TAVR). Persistence of flow turbulence results in the cleavage of high-molecular-weight von Willebrand multimers, primary haemostasis dysfunction and may favour bleedings. Recen...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1055/s-0038-1639352
更新日期:2018-05-01 00:00:00
abstract::The effect of chronic administration of ethanol to rats in a controlled regimen upon the activities of the coagulation factors II, VII, VIII, IX and X has been studied. No significant differences were found for factor II, IX and X between the ethanol-treated rats and the control group given an isocaloric diet with car...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1976-12-31 00:00:00
abstract::Fibrinolytic therapy can result in rapid lysis of deep vein thrombi (DVT), but its use is limited by the failure of many patients to respond and by the increased risk of bleeding complications in comparison with anticoagulant therapy alone. Treatment could be improved by the ability to select patients most likely to r...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1995-03-01 00:00:00
abstract::Fresh retracted clots are known to be poorly lysable by fibrinolytic agents. We have studied whether lysis of retracted clots could be enhanced by bulk transport in comparison to pure diffusion of plasma containing urokinase (400 IU/ml) into the clots. Cylindrical retracted blood clots were occlusively glued by a poly...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1992-12-07 00:00:00
abstract::Acute promyelocytic leukaemia (APL) may be associated with disseminated intravascular coagulation, as a result of increased tissue factor (TF) expression and reduced thrombomodulin (TM) expression by APL blast cells. During retinoid acid (RA)- and dibutyryl cAMP (dbcAMP)-induced differentiation of the APL cells, there...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:2001-06-01 00:00:00