Heterozygous abnormal fibrinogen Osaka III with the replacement of gamma arginine-275 by histidine has an apparently higher molecular weight gamma-chain variant.

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 the polymerization of preformed fibrin monomer which was prepared by the treatment of fibrinogen with thrombin or reptilase was also markedly defective. Propositus' fibrinogen had normal crosslinking abilities of alpha- and gamma-chains. Analysis of fibrinogen chains on sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) in the system of Laemmli only revealed the presence of abnormal gamma-chain with an apparently higher molecular weight, the presence of which was more clearly detected with SDS-PAGE of fibrin monomer obtained by thrombin treatment. Purified fragment D1 of fibrinogen Osaka III also seemed to contain an apparently higher molecular weight fragment D1 gamma remnant on Laemmli gels, which was digested faster than the normal control by plasmin in the presence of [ethylenebis(oxyethylenenitrilo)]tetraacetic acid (EGTA).

journal_name

Thromb Haemost

authors

Yoshida N,Imaoka S,Hirata H,Matsuda M,Asakura S

keywords:

subject

Has Abstract

pub_date

1992-11-10 00:00:00

pages

534-8

issue

5

eissn

0340-6245

issn

2567-689X

journal_volume

68

pub_type

杂志文章
  • Extracellular regulation of TGF-beta activity in wound repair: growth factor latency as a sensor mechanism for injury.

    abstract::The transforming growth factor-beta (TGFbeta) family of growth factors are major regulators of wound repair, scar formation, and fibrosis. One of the prominent features of TGFbeta biology is the fact that this growth factor is secreted as a latent precursor, which may be directed to and stored at specific sites in the...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:10.1160/TH04-05-0324

    authors: Brunner G,Blakytny R

    更新日期:2004-08-01 00:00:00

  • Abnormality of the N-terminal portion of von Willebrand factor in type IIA and IIC von Willebrand disease.

    abstract::We have established a new analytical method which allows the characterization of von Willebrand factor (vWF) degradation fragments in minute amounts (10 microliters) of plasma, without the need for immunopurification of vWF. Plasma vWF was hydrolysed by S aureus V-8 protease (V-8 protease) and the cleaved fragments se...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Takahashi Y,Kalafatis M,Girma JP,Meyer D

    更新日期:1988-12-22 00:00:00

  • Homozygous Pro74-->Arg mutation in the platelet glycoprotein Ibbeta gene associated with Bernard-Soulier syndrome.

    abstract::Bernard-Soulier syndrome (BSS) is an autosomal recessive bleeding disorder due to quantitative or qualitative abnormalities in the glycoprotein (GP) Ib/IX/V complex, the platelet receptor for von Willebrand factor. This complex is composed of four subunits, GPIbalpha, GPIbbeta, GPIX and GPV. We describe here the genet...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Kunishima S,Tomiyama Y,Honda S,Fukunishi M,Hara J,Inoue C,Kamiya T,Saito H

    更新日期:2000-07-01 00:00:00

  • Uremic medium disturbs the hemostatic balance of cultured human endothelial cells.

    abstract::We have investigated the ability of serum from uremic patients to modify the thrombogenic properties of the endothelium. The effects of uremic medium on the morphology of endothelial cells (ECs), and their resistance to flow was analyzed. The influence of uremic media on the reactivity of the extracellular matrix (ECM...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Serradell M,Díaz-Ricart M,Cases A,Zurbano MJ,Aznar-Salatti J,López-Pedret J,Ordinas A,Escolar G

    更新日期:2001-10-01 00:00:00

  • PAI-1 level and the PAI-1 4G/5G polymorphism in relation to risk of non-fatal myocardial infarction: results from the Stockholm Heart Epidemiology Program (SHEEP).

    abstract::This study examines the relationship between plasma Plasminogen Activator Inhibitor-1 (PAI-1) activity and the PAI-1 4G/5G polymorphism, and their association with the risk of myocardial infarction (MI). Furthermore, this study explores whether a high level of PAI-1 or whether the PAI-1 4G/5G polymorphism synergistica...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Leander K,Wiman B,Hallqvist J,Sten-Linder M,de Faire U,Stockholm Heart Epidemiology Program.

    更新日期:2003-06-01 00:00:00

  • PML/RARalpha plays a role for basal activity and retinoid-induced repression of the tissue factor promoter in acute promyelocytic leukemia cells.

    abstract::Constitutive expression of tissue factor (TF) by acute promyelocytic leukemia (APL) cells may contribute to thrombotic complications. In this study we examined the transcriptional mechanisms of all-trans retinoic acid (ATRA)-induced down-regulation of TF in the APL cell line NB4, by analysis of stable clones expressin...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1160/TH03-02-0087

    authors: Tenno T,Oberg F,Mackman N,Nilsson K,Siegbahn A

    更新日期:2003-11-01 00:00:00

  • Anticoagulation in patients with atrial fibrillation undergoing coronary stent implantation.

    abstract::In patients with atrial fibrillation (AF) undergoing coronary stent implantation, the optimal antithrombotic strategy is unclear. We evaluated whether use of oral anticoagulation (OAC) was associated with any benefit in morbidity or mortality in patients with AF, high risk of thromboembolism (TE) (CHA2DS2-VASC score ≥...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1160/TH13-04-0351

    authors: Bernard A,Fauchier L,Pellegrin C,Clementy N,Saint Etienne C,Banerjee A,Naudin D,Angoulvant D

    更新日期:2013-09-01 00:00:00

  • Studies of heparin binding to antithrombin III by crossed immunoelectrophoresis.

    abstract::The technique of crossed immunoelectrophoresis has been used to study the binding to purified antithrombin III (At III) of heparin and other mucopolysaccharides. The technique was unable to detect differences among samples of whole heparin from various manufacturers, but proved useful in studying the binding of hepari...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Barrowcliffe TW

    更新日期:1980-02-29 00:00:00

  • Procoagulant Effects of Low-Level Platelet Activation and Its Inhibition by Colchicine.

    abstract::Platelets play an important role in diseases such as cardiovascular disease and cancer, especially through their release of extracellular vesicles (EVs) and role in thrombosis. The effects of the anti-inflammatory drug colchicine on platelets are not well understood. We investigated the effect of colchicine on the rel...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0038-1636915

    authors: Reddel CJ,Pennings GJ,Curnow JL,Chen VM,Kritharides L

    更新日期:2018-04-01 00:00:00

  • F7 gene variants modulate protein levels in a large cohort of patients with factor VII deficiency. Results from a genotype-phenotype study.

    abstract::Congenital factor VII (FVII) deficiency is a rare bleeding disorder caused by mutations in F7 gene with autosomal recessive inheritance. A clinical heterogeneity with poor correlation with FVII:C levels has been described. It was the objective of this study to identify genetic defects and to evaluate their relationshi...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,多中心研究

    doi:10.1160/TH17-02-0085

    authors: Quintavalle G,Riccardi F,Rivolta GF,Martorana D,Di Perna C,Percesepe A,Tagliaferri A,Ad-Hoc Study Group.

    更新日期:2017-08-01 00:00:00

  • Shear stress regulates inflammatory and thrombogenic gene transcripts in cultured human endothelial progenitor cells.

    abstract::Shear stress has an established effect on mature endothelial cells, but less is known about how shear stress regulates endothelial progenitor cells (EPCs). In vitro expanded EPCs isolated from adult human blood represent a novel tool in regenerative vessel therapy. However, in vitro culturing may generate cells with u...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1160/TH09-12-0854

    authors: Lund T,Hermansen SE,Andreasen TV,Olsen JO,Østerud B,Myrmel T,Ytrehus K

    更新日期:2010-09-01 00:00:00

  • Angiotensin II enhances thrombosis development in renovascular hypertensive rats.

    abstract::There is an increased number of in vitro evidence that angiotensin II (Ang II) may promote thrombosis. However there are no in vivo experiments exploring the effect of Ang II on thrombus formation. In the present study we have investigated the influence of Ang II on venous thrombosis in renovascular hypertensive rats....

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1160/TH04-10-0701

    authors: Mogielnicki A,Chabielska E,Pawlak R,Szemraj J,Buczko W

    更新日期:2005-06-01 00:00:00

  • Thrombin production, inactivation and expression during open heart surgery measured by assays for activation fragments including a new ELISA for prothrombin fragment F1 + 2.

    abstract::Activation of coagulation was studied during the peri-operative period in patients undergoing cardiopulmonary bypass (CPB) surgery using activation markers which have recently become available: prothrombin fragment F1 + 2 (F1 + 2), which is a measure of total thrombin generation, and thrombin-antithrombin complex, whi...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Boisclair MD,Lane DA,Philippou H,Sheikh S,Hunt B

    更新日期:1993-08-02 00:00:00

  • Thrombolytic and pharmacokinetic properties of human tissue-type plasminogen activator variants, obtained by deletion and/or duplication of structural/functional domains, in a hamster pulmonary embolism model.

    abstract::A pulmonary embolism model in hamsters was used for the quantitative evaluation of the thrombolytic and pharmacokinetic properties of variants of tissue-type plasminogen activator (t-PA). A 25 microliters 125I-fibrin labeled human plasma clot was made in vitro and injected into the jugular vein of heparinized hamsters...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Collen D,Lijnen HR,Vanlinthout I,Kieckens L,Nelles L,Stassen JM

    更新日期:1991-02-12 00:00:00

  • Thrombolysis with an Escherichia coli-produced recombinant plasminogen activator (BM 06.022) in the rabbit model of jugular vein thrombosis.

    abstract::The recombinant plasminogen activator BM 06.022 consists of the kringle 2 and the protease domains of human t-PA and is unglycosylated because of the expression in Escherichia coli. The thrombolytic and pharmacokinetic properties as well as the hemostasis effects of BM 06.022 were investigated in the rabbit model of j...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Martin U,Fischer S,Kohnert U,Opitz U,Rudolph R,Sponer G,Stern A,Strein K

    更新日期:1991-05-06 00:00:00

  • Human platelet CD36 (GPIIIb, GPIV) binds to cholesteryl-hemisuccinate and can be purified by a simple two-step method making use of this property.

    abstract::CD36, also known as GPIIIb or GPIV, is a main protein of the platelet membrane. Accumulating evidence implicates CD36 in the pathogenesis of atherosclerosis. CD36 binds to cholesteryl-hemisuccinate coupled to agarose. This binding may be of physiologic relevance since CD36 has a long extracellular hydrophobic region a...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Kronenberg A,Grahl H,Kehrel B

    更新日期:1998-05-01 00:00:00

  • The second laminin G-type domain of protein S is indispensable for expression of full cofactor activity in activated protein C-catalysed inactivation of factor Va and factor VIIIa.

    abstract::Vitamin K-dependent protein S is a cofactor to the anticoagulant serine protease activated protein C (APC) in the proteolytic inactivation of the procoagulant, activated factor V (FVa) and factor VIII (FVIIIa). In the FVa degradation, protein S selectively accelerates the cleavage at Arg306, having no effect on the Ar...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Evenäs P,García de Frutos P,Nicolaes GA,Dahlbäck B

    更新日期:2000-08-01 00:00:00

  • Clinical use of a rapid collagen binding assay for von Willebrand factor cleaving protease in patients with thrombotic thrombocytopenic purpura.

    abstract::A simple collagen binding assay (CBA) for measuring activity of the von Willebrand factor cleaving protease in clinical samples is described, and results of fifty masked plasmapheresis samples rom patients with TTP/HUS and other diseases are presented. There was 97.5% concordance between the CBA and a multimer gel ass...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Rick ME,Moll S,Taylor MA,Krizek DM,White GC 2nd,Aronson DL

    更新日期:2002-10-01 00:00:00

  • Can Sequential Coagulation Monitoring Predict Major Haemorrhage in Extremely Low Birth Weight Infants?

    abstract:INTRODUCTION: Bleeds such as intra-ventricular (IVH) and pulmonary haemorrhage (PH) are life-threatening events in extremely low birth weight (ELBW) infants. Serial coagulation monitoring by measuring the international normalized ratio (INR) with small volume samples might facilitate early diagnosis and possibly preven...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0038-1655744

    authors: Thanhaeuser M,Binder C,Derhaschnig U,Jilma B,Kornsteiner-Krenn M,Huber-Dangl M,Repa A,Kreissl A,Berger A,Haiden N

    更新日期:2018-07-01 00:00:00

  • Do extra-platelet sources contribute to the plasma level of thrombospondin?

    abstract::Thrombospondin, a trimeric glycoprotein contained in the platelet alpha-granules, has been proposed as a marker of in vivo platelet activation. However, it is also synthesised by a range of other cells. The extraplatelet contribution to plasma levels of thrombospondin was therefore estimated by investigating the relat...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Dawes J,Pratt DA,Dewar MS,Preston FE

    更新日期:1988-04-08 00:00:00

  • Plasma D-dimer as a marker for postoperative deep venous thrombosis. A study after total hip or knee arthroplasty.

    abstract::Plasma levels of D-dimer have been found useful as a marker of deep venous thrombosis in those patients in whom thrombosis is suspected, but their usefulness in postoperative screening is less clear. We have investigated the relationship of D-dimer to deep venous thrombosis in 90 patients after total hip and knee arth...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Dunn ID,Hui AC,Triffitt PD,Crozier AE,Gregg PJ,Sinclair ME,Armstrong AL,Mitchell VE

    更新日期:1994-11-01 00:00:00

  • Contribution of polymorphisms in the endothelial protein C receptor gene to soluble endothelial protein C receptor and circulating activated protein C levels, and thrombotic risk.

    abstract::Endothelial cell protein C receptor (EPCR) enhances the generation of activated protein C (APC) by the thrombin-thrombomodulin complex. A soluble form of EPCR (sEPCR), which is generated by metalloprotease activity, is present in plasma. The distribution of sEPCR levels in healthy populations is bimodal. Previously, w...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1160/TH03-10-0657

    authors: Medina P,Navarro S,Estellés A,Vayá A,Woodhams B,Mira Y,Villa P,Migaud-Fressart M,Ferrando F,Aznar J,Bertina RM,España F

    更新日期:2004-05-01 00:00:00

  • D-dimer in local thrombolytic therapy with low doses of recombinant human tissue-type plasminogen activator (rt-PA) in patients with peripheral arterial occlusive disease.

    abstract::The generation of D-dimer was studied in the course of local thrombolytic therapy of peripheral arterial occlusions with low doses of recombinant human tissue-type plasminogen activator (rt-PA) in 7 patients. Intermittent local application of rt-PA resulted in a marked increase in D-dimer exceeding values usually seen...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Koppensteiner R,Speiser W,Minar E,Ahmadi R,Ehringer H

    更新日期:1990-10-22 00:00:00

  • Lipoprotein(a) levels and isoforms and fibrinolytic activity in postmenopause--influence of hormone replacement therapy.

    abstract::Epidemiological studies suggest that hormone replacement therapy (HRT) decreases the risk of cardiovascular disease in postmenopausal women via several mechanisms, including modifications in the fibrinolytic system and lipoprotein(a) [Lp(a)] levels. The aim of this study was to examine the influence of the levels and ...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Estellés A,Cano A,Falcó C,España F,Gilabert J,Grancha S,Aznar J

    更新日期:1999-01-01 00:00:00

  • Thrombin generation, a function test of the haemostatic-thrombotic system.

    abstract::By the use of a fluorogenic thrombin substrate and continuous calibration of each individual sample, it is now possible to obtain a thrombin generation (TG) curve (or thrombogram) in plasma, with or without platelets, in an easy routine procedure at high throughput and with an acceptable experimental error (<5%). Evid...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:

    authors: Hemker HC,Al Dieri R,De Smedt E,Béguin S

    更新日期:2006-11-01 00:00:00

  • Prediction of pulmonary embolism extent by clinical findings, D-dimer level and deep vein thrombosis shown by ultrasound.

    abstract::Pulmonary embolism (PE) may encompass a wide spectrum of severity. To determine whether clinical findings, D-dimer (DD) concentration, and deep vein thrombosis (DVT) shown by lower-limb venous compression ultrasonography (US) might predict the scintigraphic extent of PE, we studied 104 hemodynamically stable consecuti...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Galle C,Papazyan JP,Miron MJ,Slosman D,Bounameaux H,Perrier A

    更新日期:2001-11-01 00:00:00

  • Polymorphisms in the protein C gene as risk factor for venous thrombosis.

    abstract::Protein C is an important inhibitor of blood coagulation. We investigated the effect of two polymorphisms within the promoter region of the protein C gene (C/T at position 2405 and A/G at position 2418) on risk of venous thrombosis and on plasma protein C levels. In addition the combined effect of the two polymorphism...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Pomp ER,Doggen CJ,Vos HL,Reitsma PH,Rosendaal FR

    更新日期:2009-01-01 00:00:00

  • Antithrombotic activity of LB30057, a newly synthesized direct thrombin inhibitor.

    abstract::An amidrazonophenylalanine derivative, LB30057, inhibits the catalytic activity of thrombin potently by interaction with the active site of thrombin, and has high water solubility. In the present study, we evaluated the effect of LB30057 on the biological activities of thrombin at various tissues, and determined wheth...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Kang KT,Jung BI,Bae ON,Lee MY,Chung SM,Lee JY,Lee SK,Kim IC,Chung JH

    更新日期:2003-01-01 00:00:00

  • The role of actin-binding proteins in the control of endothelial barrier integrity.

    abstract::The endothelial barrier of the vasculature is of utmost importance for separating the blood stream from underlying tissues. This barrier is formed by tight and adherens junctions (TJ and AJ) that form intercellular endothelial contacts. TJ and AJ are integral membrane structures that are connected to the actin cytoske...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:10.1160/TH14-04-0298

    authors: García-Ponce A,Citalán-Madrid AF,Velázquez-Avila M,Vargas-Robles H,Schnoor M

    更新日期:2015-01-01 00:00:00

  • The HR2 haplotype of factor V: effects on factor V levels, normalized activated protein C sensitivity ratios and the risk of venous thrombosis.

    abstract::We studied the HR2 haplotype of the factor V gene in a case-control study for venous thrombosis including 474 patients with a first deep-vein thrombosis and 474 age- and sex-matched healthy controls (Leiden Thrombophilia Study, LETS). We investigated both the original His1299Arg (A4070G) polymorphism and the Met385Thr...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: de Visser MC,Guasch JF,Kamphuisen PW,Vos HL,Rosendaal FR,Bertina RM

    更新日期:2000-04-01 00:00:00