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https://www.readbyqxmd.com/read/29251640/a-novel-factor-x-mutation-cys81-by-arg-and-a-reported-factor-vii-polymorphism-arg353-replaced-by-gln-co-occured-in-a-patient
#1
Yanhui Jin, Xiaoli Cheng, Jiayong Zheng, Hong Xia, Lihong Yang, Mingshan Wang
: Coagulation factor X and factor VII (FVII) are both very important components in blood coagulation. To study the molecular pathogenic mechanism of the inherited factor X and FVII deficiency, the factor X activity (FX:C) and FVII activity were tested with one-stage clotting methods. The factor X antigen and factor FVII antigen were tested with ELISA. All the exons, intron-exon boundaries and 5',3'-flanking regions of F10 and F7 genes were amplified by PCR with direct sequencing. The ClustalX software was used to analyze the conservative property of the mutation sites...
December 14, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29246447/factor-vii-deficiency-unveiling-the-cellular-and-molecular-mechanisms-underlying-three-model-alterations-of-the-enzyme-catalytic-domain
#2
Maria Eugenia Chollet, Elisabeth Andersen, Ellen Skarpen, Christiane F Myklebust, Christian Koehler, Jens Preben Morth, Ampaiwan Chuansumrit, Mirko Pinotti, Francesco Bernardi, Bernd Thiede, Per Morten Sandset, Grethe Skretting
Activated factor (F) VII is a vitamin K-dependent glycoprotein that initiates blood coagulation upon interaction with tissue factor. FVII deficiency is the most common of the rare congenital bleeding disorders. While the mutational pattern has been extensively characterized, the pathogenic molecular mechanisms of mutations, particularly at the intracellular level, have been poorly defined. Here, we aimed at elucidating the mechanisms underlying altered FVII biosynthesis in the presence of three mutation types in the catalytic domain: a missense change, a microdeletion and a frameshift/elongation, associated with severe or moderate to severe phenotypes...
December 12, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29246306/factor-vii-induced-microrna-135a-inhibits-autophagy-and-is-associated-with-poor-prognosis-in-hepatocellular-carcinoma
#3
Kuang-Tzu Huang, I-Ying Kuo, Ming-Chao Tsai, Chun-Hsien Wu, Li-Wen Hsu, Li-Yu Chen, Chao-Pin Kung, Yu-Fan Cheng, Shigeru Goto, Yu-Wei Chou, Chao-Long Chen, Chih-Che Lin, Kuang-Den Chen
Hepatocellular carcinoma (HCC) is one of the most common and aggressive malignancies worldwide. Treatment outcomes remain poor mainly due to lack of good diagnostic/prognostic markers and limited therapeutic strategies. We previously characterized aberrant activation of the TF/FVII/PAR2 pathway, which subsequently results in decreased autophagy, as a crucial event in malignant progression of HCC. Here, we identified miR-135a as a highly upregulated miRNA in HCC in response to TF/FVII/PAR2 activation. Analyzing 103 HCC patient specimens, we confirmed that miR-135a was frequently elevated in HCC tissues with higher FVII expression compared to adjacent non-cancerous counterparts...
December 15, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/29235093/role-of-clinical-and-laboratory-parameters-for-treatment-choice-in-patients-with-inherited-fvii-deficiency-undergoing-surgical-procedures-evidence-from-the-ster-registry
#4
Matteo N D Di Minno, Mariasanta Napolitano, Alberto Dolce, Guglielmo Mariani
Perioperative bleeding is a major concern in patients with factor VII (FVII) deficiency. Evaluating data of 95 FVII-deficient patients undergoing 110 surgical procedures (61 major, 49 minor), we assessed the impact of type of surgery, bleeding phenotype and FVII coagulant activity (FVII:C) levels on perioperative replacement therapy (RT). Compared to those with higher FVII:C levels, patients with <3% FVII:C received a higher number of RT doses (8 vs. 2, P = 0·003) for a longer RT duration (3 days vs...
December 12, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29207137/in%C3%A2-vitro-expression-of-mutant-factor-vii-proteins-and-characterization-of-their-clinical-significance
#5
Amir Mashayekhi, Shirin Shahbazi, Mirdavood Omrani, Reza Mahdian
Factor VII (FVII) serves an essential role in the initiation of blood coagulation. Mutations in conserved residues within its serine protease domain may lead to dysregulated coagulation activity. The objective of the present study was to elucidate the impact of altering two conserved residues, H348R and S282R, on the functional properties of the FVII protein. The mutation‑harboring fragments were derived from genomic DNA of a FVII deficient patient. The fragments were integrated into a pcDNA vector containing FVII cDNA of HepG2 cells...
November 27, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29204261/recombinant-human-factor-viia-rfviia-in-hemophilia-mode-of-action-and-evidence-to-date
#6
REVIEW
Muriel Giansily-Blaizot, Jean-François Schved
Recombinant activated factor VII (rFVIIa) is a bypassing agent widely used both in the treatment and prevention of hemorrhagic complications due to hemophilia with inhibitor. In such cases, antihemophilic factors cannot be used. The normal physiology of factor VII/ factor VIIa (FVII/FVIIa) in the hemostatic process requires the presence of tissue factor (TF) that links to FVII leading to a FVIIa-TF complex which activates both factor X and factor IX. The therapeutic use of rFVIIa requires high amount of FVIIa...
December 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29126301/coagulation-testing-in-the-core-laboratory
#7
William E Winter, Sherri D Flax, Neil S Harris
Primary hemostasis begins with endothelial injury. VWF, produced by endothelial cells, binds to platelets and links them to subendothelial collagen. Platelet-derived ADP and thromboxane activate non-adhered platelets via their GPIIb/IIIa receptors, allowing these platelets to participate in platelet aggregation. Secondary hemostasis is initiated with the binding of factor VII to extravascular tissue factor (TF). Factors II, VII, IX and X are vitamin K-dependent factors. The role of vitamin K is to assist in the addition of gamma carboxylate groups to glutamic acids in the "GLA" domains of these factors...
November 8, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29112333/associations-of-activated-coagulation-factor-vii-and-factor-viia-antithrombin-levels-with-genome-wide-polymorphisms-and-cardiovascular-disease-risk
#8
N C Olson, L M Raffield, L A Lange, E M Lange, W T Longstreth, G Chauhan, S Debette, S Seshadri, A P Reiner, R P Tracy
ESSENTIALS: Essentials A fraction of coagulation factor VII circulates in blood as an activated protease (FVIIa). We evaluated FVIIa and FVIIa-antithrombin (FVIIa-AT) levels in the Cardiovascular Health Study. Polymorphisms in the F7 and PROCR loci were associated with FVIIa and FVIIa-AT levels. FVIIa may be an ischemic stroke risk factor in older adults and FVIIa-AT may assess mortality risk. SUMMARY: Background A fraction of coagulation factor (F) VII circulates as an active protease (FVIIa)...
November 7, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29102389/coronary-artery-stenosis-treatment-in-aging-patients-with-inherited-factor-vii-deficiency-where-do-we-stand
#9
Mariasanta Napolitano, Sergio Siragusa
Aging with rare bleeding disorders such as factor VII (FVII) deficiency poses several challenges to treatment because of the occurrence of cerebral and cardiovascular age-related comorbidities and high bleeding risks. We report a case of long-term treatment with antiplatelet agents and contemporary prophylaxis of bleeding in a woman affected by severe FVII deficiency diagnosed with symptomatic coronary artery stenosis. Information on the management of antithrombotic treatment in rare bleeding disorders is lacking and mainly limited to anecdotal reports or side effects secondary to replacement therapy...
November 1, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29075790/differential-contribution-of-tissue-factor-and-factor%C3%A2-xii-to-thrombin-generation-triggered-by-breast-and-pancreatic-cancer-cells
#10
Aurélie Rousseau, Annette K Larsen, Patrick Van Dreden, Michele Sabbah, Ismail Elalamy, Grigoris T Gerotziafas
Most cancer cells trigger thrombin generation (TG) to various extent. In the present study, we dissected the mechanisms responsible for the procoagulant activity of pancreatic adenocarcinoma cells (BXPC3), a highly thrombogenic cancer type, and breast cancer cells (MCF7), a less thombogenic tumor type. TG of normal plasma was assessed by the Thrombinoscope (CAT®) in the presence or absence of cancer cells. TG was also assessed in plasma depleted of clotting factors, in plasma spiked with tissue factor (TF) and/or procoagulant phospholipids, in plasma spiked with an anti-TF monoclonal antibody or with corn trypsin inhibitor (CTI)...
October 20, 2017: International Journal of Oncology
https://www.readbyqxmd.com/read/29035661/outcome-of-laparoscopic-ovariohysterectomy-or-ovariectomy-in-dogs-with-von-willebrand-disease-or-factor-vii-deficiency-20-cases-2012-2014
#11
Thomas P Keeshen, J Brad Case, Jeffrey J Runge, Ameet Singh, Philipp D Mayhew, Michele A Steffey, William T N Culp
OBJECTIVE To describe surgical techniques and perioperative management of dogs with von Willebrand disease (VWD) or factor VII (FVII) deficiency undergoing laparoscopic ovariohysterectomy or ovariectomy and evaluate outcomes. DESIGN Retrospective case series. ANIMALS 20 client-owned dogs with VWD (n = 16) or FVII deficiency (4). PROCEDURES Dogs with VWD or FVII deficiency that underwent laparoscopic ovariohysterectomy or ovariectomy between 2012 and 2014 were retrospectively identified via a multi-institutional review of medical records...
November 1, 2017: Journal of the American Veterinary Medical Association
https://www.readbyqxmd.com/read/28969734/cerebral-venous-sinus-thrombosis-in-a-patient-with-undiagnosed-factor-vii-deficiency
#12
Hira Qadir, Anila Rashid, Salman Naseem Adil
Factor VII (FVII) deficiency is one of the rare inherited bleeding disorders. Thrombosis has been occasionally described in inherited FVII deficiency. Here, we report a young female with undiagnosed FVII deficiency who presented with cerebral venous sinus thrombosis (CVST). Oral contraceptive pill was found to be prothrombotic risk factor. The CVSToccurred in spite of the congenital FVII deficiency indicating that no definitive antithrombotic protection is assured by this defect. Low molecular weight heparin and anti-Xa assay were found to be safe choice of anticoagulation and monitoring, respectively, in this patient...
September 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28969316/perioperative-management-of-rare-coagulation-factor-deficiency-states-in-cardiac-surgery
#13
E R Strauss, M A Mazzeffi, B Williams, N S Key, K A Tanaka
Rare bleeding disorders (RBDs) include the hereditary deficiency of fibrinogen, factor (F)II, FV, FV + FVIII, FVII, FX, FXI or FXIII. RBDs do not confer a protective effect against atheromatous plaque formation, and thus the need for cardiovascular (CV) surgery in RBD patients is expected to increase with improved healthcare access (diagnosis and management) and longevity of the population. Clinical data regarding the management of RBDs in this setting are sparse, but the perioperative care team is obliged to gain a better understanding on available biological and pharmacological hemostatic agents...
September 1, 2017: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/28956468/non-ionic-surfactant-based-vesicular-drug-delivery-system-for-topical-delivery-of-caffeine-for-treatment-of-cellulite-design-formulation-characterization-histological-anti-cellulite-activity-and-pharmacokinetic-evaluation
#14
Mahmoud H Teaima, Sally A Abdelhalim, Mohamed A El-Nabarawi, Dalia A Attia, Doaa A Helal
Cellulite is a common topographical alteration where skin acquires an orange peel or mattress appearance with alterations in adipose tissue and microcirculation. This work aims to develop and evaluate a topical niosomal gel formulae with good permeation to reach the subcutaneous fat layer. Several caffeine niosomal dispersions were prepared and incorporated into gel formulae using Carbopol 940 polymer, chemical penetration enhancers, and iontophoresis, then the prepared gels were applied onto the skin of rats and anticellulite activity of caffeine from the prepared gels compared to that of the commercial product Cellu Destock(®) was evaluated by histological study of the skin and measurement of plasma level of caffeine passing through the skin using liquid chromatography (LC/MS-MS)...
October 17, 2017: Drug Development and Industrial Pharmacy
https://www.readbyqxmd.com/read/28954939/comparison-of-resin-based-and-glass-ionomer-sealants-with-regard-to-fluoriderelease-and-anti-demineralization-efficacy-on-adjacent-unsealed-enamel
#15
Thwe Zin Ei, Yasushi Shimada, Syozi Nakashima, Maria Jacinta Rosario H Romero, Yasunori Sumi, Junji Tagami
This study compared resin-based and glass ionomer sealants with regard to their fluoride-release behavior and anti-demineralization potential on adjacent unsealed enamel surfaces. Sealant cavities prepared on bovine enamel blocks were filled with fluoride-containing resin sealants [TeethmateF-1 (TF), Clinpro(TM) (CP)], and glass ionomer sealant [Fuji VII (FVII)]. Specimens were then incubated in artificial saliva for 14 days to measure fluoride. Thereafter, demineralization was performed for 10 days, and the anti-demineralization efficacy was assessed by Swept Source Optical Coherence Tomography (SS-OCT), and cross-sectional nanohardness...
September 28, 2017: Dental Materials Journal
https://www.readbyqxmd.com/read/28944952/bypassing-agent-prophylaxis-in-people-with-hemophilia-a-or-b-with-inhibitors
#16
REVIEW
Chatree Chai-Adisaksopha, Sarah J Nevitt, Mindy L Simpson, Maissaa Janbain, Barbara A Konkle
BACKGROUND: People with hemophilia A or B with inhibitors are at high risk of bleeding complications. Infusion of bypassing agents, such as recombinant activated FVII (rFVIIa) and plasma-derived activated prothrombin complex concentrate, are suggested as alternative therapies to factor VIII (haemophilia A) or IX (haemophilia B) for individuals who no longer respond to these treatments because they develop inhibitory antibodies. The ultimate goal of treatment is to preserve the individual's joints, otherwise destroyed by recurrent bleeds...
September 25, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28924539/genetic-polymorphisms-of-hemostatic-factors-and-thrombotic-risk-in-non-bcr-abl-myeloproliferative-neoplasms-a-pilot-study
#17
R Dambrauskienė, R Gerbutavičius, R Ugenskienė, R Jankauskaitė, A Savukaitytė, R Šimoliūnienė, M Rudžianskienė, R Gerbutavičienė, E Juozaitytė
The most important complications of Philadelphianegagive (non BCR-ABL) myeloproliferative neoplasms (MPNs) are vascular events. Our aim was to evaluate the effects of single nucleotide polymorphisms (SNPs), platelet glycoproteins (GPs) (Ia/IIa, Ibα, IIb/IIIa and VI), von Willebrand factor (vWF), coagulation factor VII (FVII), β-fibrinogen, and the risk of thrombosis in patients with non BCR-ABL MPNs at the Lithuanian University of Health Sciences. Kaunas, Lithuania. Genotyping was done for 108 patients. The TT genotype of the GP Ia/IIa c...
June 30, 2017: Balkan Journal of Medical Genetics: BJMG
https://www.readbyqxmd.com/read/28921441/purification-and-autoactivation-method-for-recombinant-coagulation-factor-vii
#18
Vladimir Granovski, Marcela C C Freitas, Mario Soares Abreu-Neto, Dimas T Covas
Recombinant coagulation factor VII is a very important and complex protein employed for treatment of hemophiliac patients (hemophilia A/B) who develop inhibitors antibodies to conventional treatments (FVIII and FIX). The rFVII is a glycosylated molecule and circulates in plasma as zymogen of 50 kDa. When activated the molecule is cleaved to 20-30 kDa and has a half-life of about 3 h, needing to be processed fast and efficiently until freeze-drying. Here, we describe a very simple and fast purification sequence for rFVII using affinity FVII Select resin and a dialysis system that can be easily scaled up...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28834159/a-novel-protein%C3%A2-c-factor%C3%A2-vii-chimera-provides-new-insights-into-the-structural-requirements-for-cytoprotective-protease-activated-receptor%C3%A2-1-signaling
#19
E M Gleeson, C J McDonnell, E E Soule, O Willis Fox, H Rushe, A Rehill, O P Smith, J S O'Donnell, R J S Preston
Essentials The basis of cytoprotective protease-activated receptor 1 (PAR1) signaling is not fully understood. Activated protein C chimera (APC(FVII-82) ) was used to identify requirements for PAR1 signaling. APC(FVII-82) did not initiate PAR1 signaling, but conferred monocyte anti-inflammatory activity. APC-specific light chain residues are required for cytoprotective PAR1 signaling. SUMMARY: Background Activated protein C (APC) cell signaling is largely reliant upon its ability to mediate protease-activated receptor (PAR) 1 proteolysis when bound to the endothelial cell (EC) protein C (PC) receptor (EPCR)...
August 20, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28825659/cryochemical-synthesis-of-polymorphous-nanostructures-of-a-steroid-neurohormone
#20
Yurii Morozov, Dmitry Chistyakov, Vladimir Chernyshev, Gleb Sergeev
A new cryochemical strategy of producing nanoparticles and polymorphous nanostructures of drugs is used, which is based on the dynamic combination of high and low temperatures, gas and solid phases, and inert carrier gases. This technology is applied to the synthesis of nanoparticles of steroid neurohormone dehydroepiandrosterone (DHEA). We have optimized the conditions of synthesis of the new polymorphous DHEA structure, FVII. The molecules of DHEA in FVII structure are bound by hydrogen bonds via oxygen atoms...
August 21, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
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