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 defect responsible for the abnormal prothrombin activity was determined by the polymerase chain reaction followed by single-strand conformation polymorphism (PCR-SSCP) analysis and by DNA sequence analysis of the human prothrombin gene. Substitution of a C for an A at nucleotide 10177 in the human prothrombin gene of the proband was identified, which results in the replacement of Glu-466 by Ala. The proband and one sister were homozygous for this mutation. Both parents, as well as one brother, were found to be heterozygous for this mutation. The same amino acid substitution was previously identified to be responsible for the dysfunctional protein Prothrombin Salakta and was hypothesized to result in altered substrate specificity. Four polymorphisms were also identified in the prothrombin gene from the proband when compared to the published sequence at nucleotides 554, 4048, 4272 and 10253.
journal_name
Thromb Haemostjournal_title
Thrombosis and haemostasisauthors
Degen SJ,McDowell SA,Sparks LM,Scharrer Isubject
Has Abstractpub_date
1995-02-01 00:00:00pages
203-9issue
2eissn
0340-6245issn
2567-689Xjournal_volume
73pub_type
杂志文章abstract：:Idarucizumab, a Fab fragment directed against dabigatran, produced rapid and complete reversal of the anticoagulation effect of dabigatran in animals and in healthy volunteers. The Study of the REVERSal Effects of Idarucizumab in Patients on Active Dabigatran (RE-VERSE AD™) is a global phase 3 prospective cohort study...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,多中心研究
doi：10.1160/TH15-03-0192
更新日期：2015-07-01 00:00:00
abstract：:Thrombin and other agonists that induce secretion and aggregation in human platelets also activate phospholipase D (PLD), but the signaling cascade leading to activation of PLD in human platelets is not yet clear. We have determined that apyrase, which scavenges ADP secreted during platelet activation, is able to bloc...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1998-12-01 00:00:00
abstract：:Tissue factor (TF) plays a critical role in tumour growth and metastasis, and its enhanced release into plasma in association with cellular microparticles (MPs) has recently been associated with pathological cancer progression. We have previously demonstrated significantly elevated levels of plasma TF antigen as well ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2009-06-01 00:00:00
abstract：:Hemorrhagic diathesis and widespread microthrombosis are common in heatstroke. To assess the early stages of coagulopathy in heatstroke, thrombin-antithrombin III (TAT), fibrin monomers, plasmin-alpha 2-antiplasmin (PAP), plasminogen and D-Dimer were measured in 16 heatstroke patients (means +/- SE rectal temperature ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1996-12-01 00:00:00
abstract：:The spleen is a crucial organ in humans. However, little is known about the association of venous thromboembolism (VTE) with splenic injury and splenectomy in trauma patients. The aim of this study was to determine the subsequent risk of VTE following splenic injury and splenectomy. A nationwide retrospective cohort s...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH15-05-0381
更新日期：2016-01-01 00:00:00
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：
更新日期：1991-02-12 00:00:00
abstract：:Adhesion to collagen was investigated as a function of platelet age in rat platelets. Platelet adherence was measured using EDTA-containing platelet- rich plasma which was added to preparations of collagen fibers clamped between magnetic stirrers by recording changes in light transmission. The plot of light transmissi...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1976-11-30 00:00:00
abstract：:Mice with a total deficiency in blood coagulation Factor X (FX) were generated by targeted replacement of an 18-kb fragment of the FX gene, comprising all exons encoding the mature FX protein, with a neo(r) cassette. The genotype distribution among the offspring from heterozygous breeding pairs suggested that FX defic...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2000-02-01 00:00:00
abstract：:Patients with haemophilia A have seriously impaired thrombin generation due to an inherited deficiency of factor (F)VIII, making them form unstable fibrin clots that are unable to maintain haemostasis. Data on fibrin structure in haemophilia patients remain limited. Fibrin permeability, assessed by a flow measurement ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH13-06-0479
更新日期：2014-04-01 00:00:00
abstract：:Microparticles (MPs) are formed from platelets (PMPs), endothelial cells (EMPs) and monocytes (MMPs), and in acute myocardial infarction (MI), there is an increase of MPs in the culprit artery. In this study MPs were evaluated in whole blood in 105 patients with MI at five time-points during a two-year follow-up (FU)....
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH16-11-0837
更新日期：2017-07-26 00:00:00
abstract：:Excessive leukocyte infiltration causes severe tissue damage in a variety of inflammatory diseases. The initial step in leukocyte extravasation is mediated by selectins and oligosaccharides on their glycoconjugate ligands. Human milk is a rich source of lactose-derived oligosaccharides that are partly absorbed in the ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH04-01-0055
更新日期：2004-12-01 00:00:00
abstract：:Parameters of the fibrinolytic system were studied in a primate model where the generation of thrombin was promoted in vivo. The procoagulant stimulus used was a combination of human factor Xa in combination with phosphatidylcholine/phosphatidylserine lipid vesicles (PCPS) as the source of coagulant active phospholipi...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1990-06-28 00:00:00
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 regul...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1993-12-20 00:00:00
abstract：:Thromboembolism remains a serious problem in patients with prosthetic heart valves. Previous studies documented a number of platelet abnormalities in such patients and correlated the occurrence of thromboembolic complications with short platelet survival. We have studied platelet survival and platelet aggregation in e...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1984-10-31 00:00:00
abstract：:Vitamin K antagonists (VKAs) remain as the most prescribed drug for treatment and prevention of thrombotic disorders in many countries, despite the recent approval of the new oral anticoagulants (NOACs). Although effectiveness and safety of VKAs are tightly associated to maintaining the patient within the internationa...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH13-11-0941
更新日期：2014-09-02 00:00:00
abstract：:Unfractionated heparin is commonly used as standard therapy along with aspirin for the management of acute ischaemic syndromes. However, heparin has many limitations including a poor dose effect response and an inability to inactivate clot bound thrombin. Direct thrombin inhibitors inactivate clot bound thrombin and a...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1997-07-01 00:00:00
abstract：:Alteplase is standard therapy for patients with acute, massive pulmonary embolism. The novel plasminogen activator desmoteplase displays high fibrin specificity and selectivity for fibrinbound plasminogen. In a preclinical model desmoteplase was twice as potent with a shorter lysis time and lower reocclusion rate. We ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,多中心研究,随机对照试验
doi：
更新日期：2009-03-01 00:00:00
abstract：:A high throughput 384-well plate assay of blood function in 60 microl reactions with the fluorogenic thrombin substrate, boc-VPR-MCA, allowed for real-time monitoring of coagulation under a diverse set of reaction conditions. Using recalcified, citrated whole blood diluted 3-fold with corn trypsin inhibitor (to block ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH04-03-0154
更新日期：2004-10-01 00:00:00
abstract：:Coagulation factor III (tissue factor) is required for significant activation of factor X in the presence of factor VIIa. Factor III is a membrane protein which requires bound phospholipids for activity, and removal of lipids during purification of factor III abolishes its activity. The activity can be recovered if li...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1980-08-29 00:00:00
abstract：:Inherited severe hypoplasminogenaemia is a multisystemic disorder leading to deficient extravascular fibrinolysis. As a clinical consequence wound healing capacity of mucous membranes is markedly impaired leading to ligneous conjunctivitis and several other manifestations. Here we report the molecular genetic and clin...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH10-04-0216
更新日期：2011-03-01 00:00:00
abstract：:A complex of heparin and a quaternary ammonium compound dissolved in an organic solvent was investigated for its potential ability to prevent clotting on the surface of plastic catheters. Despite the complexing of heparin to ammonium, anticoagulant activity remained on in vitro testing. Both the heparin complex and it...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1979-05-25 00:00:00
abstract：:Vascular smooth muscle cell (VSMC) proliferation occurs in vascular obstructive events such as atherosclerosis and restenosis. We previously showed that Notch receptors are induced in smooth muscle cells during vascular remodeling. Our goal was to determine the mechanisms employed by Notch signaling to regulate prolif...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH06-04-0224
更新日期：2006-09-01 00:00:00
abstract：:The pathogenesis of abdominal aortic aneurysm (AAA) involves a central component of chronic inflammation which is predominantly mediated by myeloid cells. We hypothesized that the local inflammatory activity may be reflected in systemic alterations of neutrophil and monocyte populations as well as in soluble factors o...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1055/s-0039-1679939
更新日期：2019-05-01 00:00:00
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：
更新日期：2009-01-01 00:00:00
abstract：BACKGROUND: Evaluation of thromboembolic risk is essential in anticoagulated atrial fibrillation (AF) patients. The CHA2DS2-VASc score is largely validated and recommended by most guidelines. The GARFIELD-AF Stroke score has been proposed as an alternative risk score. METHODS: We analyzed warfarin-treated patients fro...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1055/a-1333-4448
更新日期：2020-12-09 00:00:00
abstract：:Nipah virus (NiV) is a highly pathogenic paramyxovirus that was first isolated in 1999 during an outbreak in Malaysia. In contrast to other paramyxoviruses NiV infects many mammalian species. Because of its zoonotic potential, the high pathogenicity and the lack of therapeutic treatment, NiV was classified as a biosaf...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：10.1160/TH09-05-0310
更新日期：2009-12-01 00:00:00
abstract：:Sulodexide, a highly purified glycosaminoglycan, was investigated for treatment of venous leg ulcers. Patients (n = 235) undergoing local treatment including wound care and compression bandaging, were randomised to receive either sulodexide or matching placebo for three months. Primary study endpoint was complete ulce...
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：2002-06-01 00:00:00
abstract：:Osteoprotegerin (OPG) regulates bone metabolism by reducing the activation of osteoclasts, but may also be involved in blood vessel calcification and atherosclerosis. Within endothelial cells OPG is stored in Weibel-Palade bodies (WPBs). Blood kinetics of OPG are essentially unknown. We aimed to assess these using two...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1055/s-0040-1712448
更新日期：2020-07-01 00:00:00
abstract：:We have previously suggested that the human homologue of the Drosophila transient receptor potential protein, TRPC1, is involved in conducting store-operated Ca2+ entry (SOCE) in human platelets since an antibody raised against the pore-forming region of TRPC1 inhibited SOCE. Here we have investigated plasma membrane ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH05-06-0391
更新日期：2005-10-01 00:00:00
abstract：:We analyzed the protein C gene (PROC) with the denaturing gradient gel electrophoresis (DGGE) scanning strategy in a series of 129 patients with suspected protein C (PC) deficiency (93 with low plasma PC levels and 36 with borderline level). At least one sequence variation was found in 104 of the 129 patients. Thirty-...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2000-01-01 00:00:00
