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https://www.readbyqxmd.com/read/28799584/molecular-determinants-involved-in-differential-behaviour-between-soluble-tissue-factor-and-full-length-tissue-factor-towards-factor-viia
#1
Ramesh Prasad, Prosenjit Sen
During blood-coagulation, the transmembrane protein tissue factor (TF) binds to its ligand, factor (F)VII, activating and allosterically modifying it to form a mature active binary complex (TF-FVIIa). Although the extracellular domain of TF (sTF) can bind to FVII, it fails to activate it. Binding of TF with FVIIa only partially enhances FVIIa proteolytic activity. Our previous kinetic study revealed that sTF has a lower binding capacity with FVIIa compared to membrane bound full-length (fl)TF. The reason behind this incapability of FVII activation and reduced catalytic activity remains unexplored due to the lack of an flTF crystal structure...
August 11, 2017: Physical Chemistry Chemical Physics: PCCP
https://www.readbyqxmd.com/read/28726978/analysis-of-the-substrate-specificity-of-factor-vii-activating-protease-fsap-and-design-of-specific-and-sensitive-peptide-substrates
#2
Emrah Kara, Dipankar Manna, Geir Åge Løset, Eric L Schneider, Charles S Craik, Sandip Kanse
Factor VII (FVII) activating protease (FSAP) is a circulating serine protease that is likely to be involved in a number of disease conditions such as stroke, atherosclerosis, liver fibrosis, thrombosis and cancer. To date, no systematic information is available about the substrate specificity of FSAP. Applying phage display and positional scanning substrate combinatorial library (PS-SCL) approaches we have characterised the specificity of FSAP towards small peptides. Results were evaluated in the context of known protein substrates as well as molecular modelling of the peptides in the active site of FSAP...
July 20, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28684918/genotype-and-phenotype-correlation-in-intracranial-hemorrhage-in-neonatal-factor-vii-deficiency-among-thai-children
#3
Chanchai Traivaree, Chalinee Monsereenusorn, Arunotai Meekaewkunchorn, Premsak Laoyookhong, Saranya Suwansingh, Boonchai Boonyawat
Congenital factor VII (FVII) deficiency is a rare inherited coagulopathy. The clinical manifestations and clinical findings vary widely, ranging from asymptomatic to life-threatening bleeding, including intracranial hemorrhage (ICH), with prolonged prothrombin time, normal partial thromboplastin time and normal platelet counts, which are confirmed by the low level of FVII assay. Treatment consists of fresh frozen plasma (FFP), prothrombin complex concentrates (PCCs), and recombinant activated FVII to treat bleeding and prophylactic therapy...
2017: Application of Clinical Genetics
https://www.readbyqxmd.com/read/28651975/production-of-coagulation-factor-vii-in-human-cell-lines-sk-hep-1-and%C3%A2-hkb-11
#4
Marcela Cristina Corrêa de Freitas, Aline de Sousa Bomfim, Amanda Mizukami, Virgínia Picanço-Castro, Kamilla Swiech, Dimas Tadeu Covas
Recombinant factor VII (rFVII) is the main therapeutic choice for hemophilia patients who have developed inhibitory antibodies against conventional treatments (FVIII and FIX). Because of the post-translational modifications, rFVII needs to be produced in mammalian cell lines. In this study, for the first time, we have shown efficient rFVII production in HepG2, Sk-Hep-1, and HKB-11 cell lines. Experiments in static conditions for a period of 96 h showed that HepG2-FVII produced the highest amounts of rhFVII, with an average of 1843 ng/mL...
September 2017: Protein Expression and Purification
https://www.readbyqxmd.com/read/28604077/-activated-factor-vii-31-years-experience-on-clinical-grounds
#5
REVIEW
Miklós Udvardy
The author provides an overview of the use of recombinant activated FVII (rFVIIa, Novoseven), which is used over 30 years, based upon international publications and also on some modest own experience. Standard, approved indications (inhibitory cases, Glanzmann thrombasthenia, prophylaxis experience) are in the focus of this paper, emphasizing the specially rapid and efficacious way of Novoseven therapy, drawing attention to excellent safety issues regarding very low immunogenicity along with low number of thrombogenic complications...
June 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28576889/blood-coagulation-system-in-patients-with-chronic-kidney-disease-a-prospective-observational-study
#6
Meng-Jie Huang, Ri-Bao Wei, Yang Wang, Ting-Yu Su, Ping Di, Qing-Ping Li, Xi Yang, Ping Li, Xiang-Mei Chen
OBJECTIVES: Thromboembolic events are the major factor affecting the prognosis of patients with chronic kidney disease (CKD). Haemostatic alterations are possible causes of these complications, but their roles remain poorly characterised. In the prospective observational study, we investigated the entire coagulation process in patients with CKD to elucidate the mechanisms of their high thromboembolic risk. METHODS: A total of 95 patients with CKD and 20 healthy controls who met the inclusion criteria were consecutively recruited from September 2015 to March 2016...
June 1, 2017: BMJ Open
https://www.readbyqxmd.com/read/28570120/characterization-of-the-coagulation-profile-in-children-with-liver-disease-and-extrahepatic-portal-vein-obstruction-or-shunt
#7
William Beattie, Maria Magnusson, Winita Hardikar, Paul Monagle, Vera Ignjatovic
INTRODUCTION: Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. MATERIALS AND METHODS: We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children...
June 1, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28544373/genetic-markers-for-inherited-thrombophilia-are-associated-with-fetal-growth-retardation-in-the-population-of-central-russia
#8
Evgeny Reshetnikov, Oksana Zarudskaya, Alexey Polonikov, Olga Bushueva, Valentina Orlova, Evgeny Krikun, Volodymyr Dvornyk, Mikhail Churnosov
AIM: The aim of this study was to examine the role of hereditary thrombophilia in the development of fetal growth retardation (FGR) in the population of Central Russia. METHODS: The case-control study sample included 497 women in the third trimester of pregnancy recruited during 2009-2013. The participants were enrolled into two groups: patients with FGR (n = 250) and controls without FGR (n = 247). The participants were genotyped for four genetic markers of hereditary thrombophilia: factor V Leiden (G > A FV, rs6025), prothrombin (G > A FII, rs1799963), factor VII (G > A FVII, rs6046), and fibrinogen (G > A FI, rs1800790)...
May 19, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28496540/recombinant-factor-viia-use-for-endoscopic-retrograde-cholangiopancreatography-with-sphincterotomy-in-a-patient-with-choledocholithiasis-and-unusual-coagulopathy
#9
Molham Abdulsamad, Pavithra Reddy, Suvarna Guvvala, Anil Dev
Endoscopic retrograde cholangiopancreatography (ERCP) is a procedure that combines the use of endoscopy and fluoroscopy to diagnose and treat pancreaticobiliary disorders. The risks of ERCP include pancreatitis, infection, bleeding and perforation. Bleeding during ERCP typically develops after sphincterotomy, hence patients should be screened and tested for coagulopathy before undergoing ERCP. Coagulopathy is a major risk factor for ERCP-related bleeding. Inherited factor VII (FVII) deficiency is a rare autosomal recessive hemorrhagic disorder that can lead to significant coagulopathy and severe bleeding if not appropriately recognized and treated preoperatively...
April 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28492702/low-plasma-fvii-c-and-activated-fvii-as-predictive-markers-for-overt-disseminated-intravascular-coagulation
#10
Surapong Lertthammakiat, Nattachai Anantasit, Usanarat Anurathapan, Nongnuch Sirachainan, Praguywan Kadegasem, Ampaiwan Chuansumrit
In sepsis, binding of factor VII (FVII:C) and activated factor VII (FVIIa) with tissue factor is the key step of coagulation resulting in disseminated intravascular coagulation (DIC). We conducted a prospective cohort study among 47 septic patients, aged 8 months to 18.8 years. They were initially divided into three groups of no DIC (n=27), non-overt DIC (n=14) and overt DIC (n=6). Blood samples were collected at 0, 24 and 48 hours (h) after the onset of sepsis. At the onset of sepsis, FVII:C tended to be lower in the non-overt DIC [median 57 % (interquartile range [IQR] 41-80)] and overt DIC groups [33 % (23-52)] than that in the no DIC group [65 % (44-87)]...
July 26, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28483635/crystal-structure-of-tissue-factor-in-complex-with-antibody-10h10-reveals-the-signaling-epitope
#11
Alexey Teplyakov, Galina Obmolova, Thomas J Malia, Bingyuan Wu, Yonghong Zhao, Susann Taudte, G Mark Anderson, Gary L Gilliland
Tissue factor (TF) initiates the extrinsic pathway of blood coagulation through sequential binding and activation of coagulation factors VII (FVII) and X (FX). In addition, through activation of G-protein-coupled protease activated receptors (PARs) TF induces cell signaling that is related to cancer, angiogenesis and inflammation. Monoclonal antibodies (mAbs) proved to be a useful tool for studying the interplay between TF signaling and coagulation. MAb 10H10 is unique in that it blocks the signaling pathway and thus inhibits angiogenesis and tumor growth without interfering with coagulation...
May 5, 2017: Cellular Signalling
https://www.readbyqxmd.com/read/28462681/warfarin-management-in-the-setting-of-fvii-deficiency-and-mechanical-circulatory-support
#12
Christin DeStefano, Katelyn Sylvester, Jean Connors, Farooq Sheikh, Joseph Catlett
No abstract text is available yet for this article.
April 1, 2017: Vascular Medicine
https://www.readbyqxmd.com/read/28447100/f7-gene-variants-modulate-protein-levels-in-a-large-cohort-of-patients-with-factor-vii-deficiency-results-from-a-genotype-phenotype-study
#13
Gabriele Quintavalle, Federica Riccardi, Gianna Franca Rivolta, Davide Martorana, Caterina Di Perna, Antonio Percesepe, Annarita Tagliaferri
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 relationships with phenotype in a large cohort of patients with FVII:C<50 %. One hundred twenty-three probands were genotyped for F7 mutations and three polymorphic variants and classified according to recently published clinical scores...
August 1, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28440004/safety-and-effectiveness-of-room-temperature-stable-recombinant-factor-viia-in-patients-with-haemophilia-a-or-b-and-inhibitors-results-of-a-multinational-prospective-observational-study
#14
K Kavakli, F Demartis, M Karimi, P Eshghi, D Neme, H Chambost, L Sommer, M Zak, G Benson
INTRODUCTION: A room temperature stable formulation of recombinant activated factor VII (NovoSeven(®) ), allowing convenient storage and therefore improved treatment access, has been developed. Bioequivalence to the previous NovoSeven(®) was demonstrated in healthy humans, leading to European approval (2008). Although no confirmed cases of neutralising antibodies to rFVIIa in patients with haemophilia A or B have been observed with the original formulation, changes in formulation or storage condition may alter immunogenicity...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28430802/the-quality-changes-in-fresh-frozen-plasma-of-the-blood-donors-at-high-altitude
#15
Zongkui Wang, Hua Liu, Miaomiao Dou, Xi Du, Jijun Hu, Na Su, Ya Wang, Rong Zhang, Changqing Li
OBJECTIVE: According to the international guidelines, fresh frozen plasma (FFP) is unanimously used to treat coagulation disorders. The quality of FFP is critical for the clinical transfusion. Till now, few studies have integratedly evaluated the differences of FFP from blood donors at between high altitude (HA) and low altitude (LA). Besides, there were no special quality standards for HA FFP in China. MATERIALS AND METHODS: Up to 41 HA (Lhasa, 3700 m) and 46 LA (Chengdu, 500 m) blood donors were included in our study to estimate the differences of FFP from HA and LA blood donors...
2017: PloS One
https://www.readbyqxmd.com/read/28417928/potential-coagulation-factor-driven-pro-inflammatory-responses-in-ovarian-cancer-tissues-associated-with-insufficient-o%C3%A2-and-plasma-supply
#16
REVIEW
Shiro Koizume, Yohei Miyagi
Tissue factor (TF) is a cell surface receptor for coagulation factor VII (fVII). The TF-activated fVII (fVIIa) complex is an essential initiator of the extrinsic blood coagulation process. Interactions between cancer cells and immune cells via coagulation factors and adhesion molecules can promote progression of cancer, including epithelial ovarian cancer (EOC). This process is not necessarily advantageous, as tumor tissues generally undergo hypoxia due to aberrant vasculature, followed by reduced access to plasma components such as coagulation factors...
April 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28409836/platelet-derived-microparticles-regulates-thrombin-generation-via-phophatidylserine-in-abdominal-sepsis
#17
Yongzhi Wang, Su Zhang, Lingtao Luo, Eva Norström, Oscar Ö Braun, Matthias Mörgelin, Henrik Thorlacius
Sepsis is associated with dysfunctional coagulation. Recent data suggest that platelets play a role in sepsis by promoting neutrophil accumulation. Herein, we show that cecal ligation and puncture (CLP) triggered systemic inflammation, which is characterized by formation of IL-6 and CXC chemokines as well as neutrophil accumulation in the lung. Platelet depletion decreased neutrophil accumulation, IL-6 and CXC chemokines formation in septic lungs. Depletion of platelets increased peak thrombin formation and total thrombin generation (TG) in plasma from septic animals...
April 14, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28407450/finding-the-optimal-dose-of-vitamin-k1-to-treat-vitamin-k-deficiency-and-to-avoid-anaphylactoid-reactions
#18
Yan-Ni Mi, Na-Na Ping, Bo Li, Xue Xiao, Yan-Bing Zhu, Lei Cao, Jian-Kang Ren, Yong-Xiao Cao
Vitamin K1 injection induces severe dose-related anaphylactoid reactions and overdose for the treatment of vitamin K deficiency. We aimed to find an optimal and small dose of vitamin K1 injection to treat vitamin K deficiency and avoid anaphylactoid reactions in animal. Rats were administered a vitamin K-deficient diet and gentamicin to establish vitamin K deficiency model. Behaviour tests were performed in beagle dogs to observe anaphylactoid reactions. The results showed an increased protein induced by vitamin K absence or antagonist II (PIVKA-II) levels, a prolonging of prothrombin time (PT) and activated partial thromboplastin time (APTT) and a decrease in vitamin K-dependent coagulation factor (F) II, VII, IX and X activities in the model group...
April 13, 2017: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/28302935/factor-x-deficiency-with-heterozygous-mutations-of-novel-p-g435s-and-known-p-g244r-in-a-patient-presenting-with-severe-umbilical-hemorrhage
#19
Yoko Matsuo, Tatsuki Mizuochi, Miho Mitsuo, Shinichiro Nakagawa, Shuichi Ozono, Koichiro Ueda, Yoko Sogabe, Ritsuko Seki, Kenji Soejima, Takashi Okamura, Yushiro Yamashita
A 10-day-old male patient was referred to our hospital with severe umbilical bleeding. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were prominently prolonged. Plasma coagulation factor X (FX) activity and antigen levels were 1% and 0.6%, respectively. A DNA sequence analysis of his leukocytes revealed a compound heterozygous state; known Gly244 to Arg (p.G244R) in exon 6 and a novel mutation of Gly 435 to Ser (p.G435S) in exon 8. A pedigree analysis showed that p.G244R originated from the paternal side, while p...
March 15, 2017: Kurume Medical Journal
https://www.readbyqxmd.com/read/28263027/coagulation-factors-anticoagulant-proteins-and-plasminogen-in-mexican-older-adults
#20
E Hernández Zamora, L O González-Espinosa, C Zavala-Hernández, E Rosales-Cruz, E Reyes-Maldonado
INTRODUCTION: Hemostasis protects upon the occurrence of vascular endothelial damage, with involving of different factors. The interaction of these factors in older adults is poorly known, and has been associated with different disorders. Therefore, we determined the activity of coagulation factors (CF), anticoagulant proteins (AP), and plasminogen (Plg), as well as the frequency of deficiencies of these proteins in a population of healthy Mexican older adults (OA). METHODS: CF (I, II, V, VII, VIII, IX, X, and XI y XII), AP [protein C (PC), protein S (PS), and antithrombin (AT)], and Plg were determined from 244 plasma samples of OA using commercial kits in a coagulometer ACL Elite Pro...
March 6, 2017: International Journal of Laboratory Hematology
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