Abstract：
:The molecular basis of Factor V deficiency has been defined in few patients only. We report a homozygous nucleotide change (G6395A) in two Tunisian probands with Factor V deficiency and bleeding episodes. This substitution results in the replacement of an arginine (R) by a histidine (H) in amino acid position 2074, located in the Factor V C2-domain. Mutations in this protein domain have not previously been described. Several lines of evidence support that this sequence variant is indeed disease causing: 1) Crystal structures of Factor V and molecular C2-domain modeling studies of H2074 suggest that the conserved R2074 is required for correct folding; 2) Structure-function studies of selective Factor V mutants (R2074A) demonstrate the importance of R2074 for structural stability of the Factor V C2-domain and for cofactor activity (1); 3) In Factor VIII, point mutations in codon 2209, which corresponds to position 2074 in Factor V, cause hemophilia A.
journal_name
Thromb Haemostjournal_title
Thrombosis and haemostasisauthors
Schrijver I,Houissa-Kastally R,Jones CD,Garcia KC,Zehnder JLkeywords:
subject
Has Abstractpub_date
2002-02-01 00:00:00pages
294-9issue
2eissn
0340-6245issn
2567-689Xjournal_volume
87pub_type
杂志文章,评审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：:Despite reports that Tris [tris (hydroxymethyl)aminomethane] affects platelets, it is often used to buffer suspending media. Human or rabbit platelets were washed and resuspended in Tyrode solution containing apyrase and 0.35% albumin. Addition of 15 mM Tris partially inhibited primary aggregation induced by 10 microM...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1984-04-30 00:00:00
abstract：:Laboratory diagnosis of antiphospholipid antibodies is important in patients with clinical features of the antiphospholipid syndrome, such as thrombosis and fetal loss. We have developed a novel method for the detection of antiphospholipid antibodies using flow cytometry. Anionic phospholipids cardiolipin, phosphatidy...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1993-10-18 00:00:00
abstract：:The gel-chromatography of the beta-alanine precipitate of human plasma and of purified fibrinogen shows the presence of different classes of fibrinogen complexes. A first class of complexes is detectable in correspondence with the ascending branch of the fibrinogen peak ("shoulder" complexes or high molecular weight f...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1055/s-0038-1657059
更新日期：1979-07-01 00:00:00
abstract：BACKGROUND:Measurements of prothrombin fragment 1+2 (F1+2), thrombin antithrombin III complexes (TAT) and D-dimer plasma levels have been proposed as non-invasive screening tests to exclude postoperative deep venous thrombosis (DVT). We investigated the diagnostic efficacy of these coagulation activation markers to rul...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：
更新日期：1998-03-01 00:00:00
abstract：:[This corrects the article DOI: 10.1160/TH14-06-0547.]. ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,已发布勘误
doi：10.1160/TH17040001
更新日期：2017-04-03 00:00:00
abstract：:Calibration of local standards for ristocetin cofactor activity of von Willebrand's factor (vWF:Rcof) against reference preparations is required to achieve a better standardization of this measurement. We have observed that in calibration assays (multiple line parallel bioassay) for vWF:Rcof, measured aggregometricall...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1987-12-18 00:00:00
abstract：:The influence of ionic and non-ionic contrast media (CM) on platelet and leukocyte activation and platelet-leukocyte crosstalk was investigated in hirudinized whole blood. The blood was incubated with and without the ionic CM ioxaglate and the nonionic CM iodixanol at 37 degrees C for 5 min, without or with stirring. ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH04-12-0809
更新日期：2005-05-01 00:00:00
abstract：:Ultra-low-molecular-weight heparins (ULMWHs) with better efficacy and safety ratios are under development; however, there are few structural data available. The main structural features and molecular weight of ULMWHs were studied and compared to enoxaparin. Their monosaccharide composition and average molecular weight...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH12-11-0795
更新日期：2013-03-01 00:00:00
abstract：:The characterization of mutations in von Willebrand disease provides useful insight into the synthesis, structure, and function of von Willebrand factor. This growing body of information has prompted a reclassification of vWD types that is intended to reflect distinct pathophysiologic mechanisms. Despite this apparent...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1995-07-01 00:00:00
abstract：:D-dimer, a degradation product of fibrin, is being increasingly used as a marker and prognostic factor in various thrombotic diseases. Previous reports have shown that obstructive sleep apnea is associated with platelet activation and hypercoagulability. The aim of the study was to assess the potential role of the pla...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2005-09-01 00:00:00
abstract：:Using the Northern blot technique, we screened 6 human hepatoma cell lines to investigate the regulation mechanism of heparin cofactor II (HC II) biosynthesis. We found that HuH-7 and Hep G2 cells constitutively expressed the HC II gene. In conditioned medium, HuH-7 cells constantly produced HC II that was functionall...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1996-02-01 00:00:00
abstract：:A collaborative study has been organised by NIBSC to examine the performance of FIXa assays between laboratories, and to investigate the need for a standard. Ampoules of 3 materials, one monocomponent concentrate (coded C) and 2 different preparations of purified human FIXa (one proposed reference preparation, coded A...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1996-12-01 00:00:00
abstract：:Over the last few years methods have been developed to assess appearance of thrombin during blood clotting in a clinical setting. This can be achieved either by measurement of the specific thrombin markers or by analysis of the thrombin generation kinetics. Thrombin markers rise following coronary occlusion and, surpr...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1995-07-01 00:00:00
abstract：:Despite great efforts in stroke research, disability and recurrence rates in ischaemic stroke remain unacceptably high. To address this issue, one potential target for novel therapeutics is the glycoprotein von Willebrand factor (vWF), which increases in thrombogenicity especially under high shear rates as it bridges ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：10.1055/s-0038-1648251
更新日期：2018-06-01 00:00:00
abstract：:Myocardial infarction results from a platelet-rich occlusive coronary thrombus. Platelet membrane glycoprotein IIb/IIIa plays an important role in platelet adhesion and aggregation. Two polymorphisms of the gene encoding the IIIa subunit. PLA1 and PLA2, have been identified. We investigated the frequency of these poly...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章
doi：
更新日期：1998-08-01 00:00:00
abstract：:Congenitally abnormal fibrinogen Osaka III with the replacement of gamma Arg-275 by His was found in a 38-year-old female with no bleeding or thrombotic tendency. Release of fibrinopeptide(s) by thrombin or reptilase was normal, but her thrombin or reptilase time in the absence of calcium was markedly prolonged and th...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1992-11-10 00:00:00
abstract：:alpha-Granule protein storage is important for producing platelets with normal haemostatic function. The low to undetectable levels of several megakaryocyte-synthesized alpha-granule proteins in normal plasma suggest megakaryocytes are important to sequester these proteins in vivo. alpha-Granule protein storage in vit...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH03-02-0125
更新日期：2003-11-01 00:00:00
abstract：:Nuclear DNA-binding protein high mobility group box 1 (HMGB1) acts as a late mediator of severe vascular inflammatory conditions, such as sepsis. Activated factor X (FXa) is an important player in the coagulation cascade responsible for thrombin generation, and it influences cell signalling in various cell types by ac...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH14-03-0233
更新日期：2014-10-01 00:00:00
abstract：:Experiments were carried out to characterize the specificity of uptake of tPA in rat liver cells. Endocytosis in liver endothelial cells of the native carbohydrate variants of tissue plasminogen activator (tPA), and tPA inactivated by diisopropyl fluorophosphate was found to be competitive, suggesting that the determi...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1988-06-16 00:00:00
abstract：:Defibrotide is an antithrombotic drug which enhances prostacyclin production and activates fibrinolytic system. The aim of this study was to investigate the improvement of walking distance in patients with intermittent claudication treated with defibrotide. DICLIS was a double blind, placebo-controlled study which inc...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：
更新日期：2000-05-01 00:00:00
abstract：:Measurement of heparin ex vivo is usually with reference to standard curve prepared with a "spiked" normal human plasma pool (NHP). When the calibration curve was prepared by addition of heparin to whole blood before plasma separation, although the linear relationship was maintained the slope was increased in comparis...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1989-04-25 00:00:00
abstract：:Cystic fibrosis (CF) is characterized by a persistent inflammatory state, which can be secondary to chronic pulmonary infection and may affect vascular endothelium. We measured circulating levels of von Willebrand factor (vWF), tissue-plasminogen activator (t-PA), and P-selectin in 20 CF patients and 20 healthy subjec...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2001-12-01 00:00:00
abstract：:In vitro, concentration-dependent effects of rt-PA on a range of coagulation and fibrinolytic assays in thawed plasma samples were investigated. In absence of a fibrinolytic inhibitor, 2 micrograms rt-PA/ml blood (3.4 micrograms/ml plasma) caused prolongation of clotting time assays and decreases of plasminogen (to 44...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1987-10-28 00:00:00
abstract：:Cysteinyl leukotrienes (i.e. LTC4, LTD4), produced by activated leukocytes or by transcellular metabolism may act at different levels on vascular smooth muscle cells (VSMC) during inflammatory process or atherosclerosis. We studied the effect of LTC4, LTD4, and LTE4 on the in vitro proliferation of rat VSMC, measured ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1996-07-01 00:00:00
abstract：:We have identified a patient with a dysfunctional prothrombin that we have designated Prothrombin Frankfurt. The proband was characterized by a prothrombin activity level of 13% and 20% compared to normal controls using two different assays with a normal prothrombin antigen level of 91% of normal controls. The genetic...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1995-02-01 00:00:00
abstract：:To study the predictive value of coagulation markers concerning clinical outcome, prothrombin fragment F1.2 (F1.2), fibrin monomer antigen (FM), D-Dimer (DD), and fibrinogen were measured in plasma samples drawn 2 and 7 days after acute myocardial infarction (AMI) in 314 consecutive patients randomized in a clinical t...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：1999-05-01 00:00:00
abstract：:We determined factor VII antigen (FVIIag) levels in 705 healthy blood donors ranging in age from 17 to 79 years using a two-site solid-phase enzyme immunoassay developed in our laboratory. The mean (+/- SD) FVIIag level for the total population was 102 +/- 31%. FVIIag levels for men (n = 375) and women (n = 330) were ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1994-07-01 00:00:00
abstract：:Treatment of bleeding in patients with Glanzmann's thrombasthenia (GT) can be hampered by iso-antibodies against the αIIbβ3 integrin, which cause rapid clearance of transfused donor platelets. Type 1 GT patients with a total absence of αIIbβ3 from the platelet surface are known to be susceptible to form such isoantibo...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH15-12-0982
更新日期：2016-08-01 00:00:00
abstract：:The fibrin-specificity and procoagulant effects of recombinant staphylokinase (Sak42D) were compared with those of recombinant tissue-type plasminogen activator (rt-PA) in patients with acute myocardial infarction. Plasma samples were obtained at baseline and at 25 and 90 min, from 24 patients who were randomly assign...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：1996-12-01 00:00:00
