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Coagulation Factor

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https://www.readbyqxmd.com/read/28646206/crispr-cas9-mediated-genome-editing-via-postnatal-administration-of-aav-vector-cures-haemophilia-b-mice
#1
Tsukasa Ohmori, Yasumitsu Nagao, Hiroaki Mizukami, Asuka Sakata, Shin-Ichi Muramatsu, Keiya Ozawa, Shin-Ichi Tominaga, Yutaka Hanazono, Satoshi Nishimura, Osamu Nureki, Yoichi Sakata
Haemophilia B, a congenital haemorrhagic disease caused by mutations in coagulation factor IX gene (F9), is considered an appropriate target for genome editing technology. Here, we describe treatment strategies for haemophilia B mice using the clustered regularly interspaced short palindromic repeat (CRISPR)/Cas9 system. Administration of adeno-associated virus (AAV) 8 vector harbouring Staphylococcus aureus Cas9 (SaCas9) and single guide RNA (sgRNA) to wild-type adult mice induced a double-strand break (DSB) at the target site of F9 in hepatocytes, sufficiently developing haemophilia B...
June 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28645365/complexity-of-immune-responses-to-aav-transgene-products-example-of-factor-ix
#2
REVIEW
Roland W Herzog
After two decades of research, in vivo gene transfer with adeno-associated viral (AAV) vectors has now resulted in successful treatments and even cures for several human diseases. However, the potential for immune responses against the therapeutic gene products remains one of the concerns as this approach is broadened to more patients, diverse diseases, and target organs. Immune responses following gene transfer of coagulation factor IX (FIX) for the treatment of the bleeding disorder hemophilia B has been extensively investigated in multiple animal models...
May 29, 2017: Cellular Immunology
https://www.readbyqxmd.com/read/28645246/igmk-paraprotein-from-gammopathy-patient-can-bind-to-cardiolipin-and-interfere-with-coagulation-assay-a-case-report
#3
Xin-Yao Wu, Yu-Feng Yin, Jia-Lin Teng, Li-Wei Zhang, Cheng-de Yang
BACKGROUND: The monoclonal gammopathies are a group of plasma-cell proliferative disorders characterized by the secretion of monoclonal immunoglobulin (M protein or paraprotein). Some rare cases have revealed the specific affinity of paraprotein as autoantibody. Here we report a patient with monoclonal gammopathy of undetermined significance (MGUS) accompanied by a remarkable increase of anticardiolipin antibody (aCL) and an extensively decreased coagulation factor activity, however, without any clinical signs of antiphospholipid syndrome (APS) and bleeding...
June 23, 2017: BMC Immunology
https://www.readbyqxmd.com/read/28643538/do-methodological-differences-account-for-the-current-controversy-on-tissue-factor-expression-in-platelets
#4
Marta Brambilla, Laura Rossetti, Chiara Zara, Paola Canzano, Peter L A Giesen, Elena Tremoli, Marina Camera
Tissue factor (TF), the key activator of the blood coagulation cascade and of thrombus formation, is also expressed by circulating human platelets. Despite the documented in-depth characterization of platelet TF carried out in the past 15 years, some authors still fail to identify TF in platelets, especially when assessment in platelet-rich plasma (PRP) or washed platelets is carried out. This study aims to extend the characterization of the subset of TF-positive platelets in PRP from healthy subjects and to verify how different centrifugation forces, used to prepare the PRP, could affect the analysis of TF-positive platelets...
June 23, 2017: Platelets
https://www.readbyqxmd.com/read/28641667/-research-progress-on-genetic-factors-and-hemophilia-a-clotting-factor-inhibitor-review
#5
Zheng-Bin Hu, Xin Sun, Li-Ya He
Hemophilia A (Hemophilia A, HA) is an X-linked recessive hereditary coagulation function disorder, the deficiency and dysfunction of blood coagulation were caused by the mutations of gene encoding clotting factor VIII. The treatment of hemophilia A still depends on the replacement therapy with blood coagulation factor. However, the repeated infusion of clotting factor will produce the neutralizing antibody against FVIII, then resulting in one of the serious complications. The reports on the incidence of inhibitor are different at home and abroad...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28641658/-effects-of-different-storage-time-and-temperature-on-coagulation-function-of-fresh-frozen-plasma-after-thawing
#6
Xiao-Yun Gao, Xin-Hua Wang
OBJECTIVE: To investigate the coagulation function changes of fresh frozen plasma storaged at different storage time and temperature after thawing. METHODS: Forty unit of fresh frozen plasma were collected, and thawed at 37 °C for 25 minutes. Each unit was divided into 2 halves: one was stored at (4±2)°C for 72 hours and the other one was stored at (25±2) °C for 72 hours. At the time point of 0, 4, 24, 48 and 72 h, thromboela-stogram TEG was measured for all samples...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28640782/perioperative-venous-thromboembolism-a-review
#7
Ronald J Gordon, Frederick W Lombard
Venous thromboembolism (VTE) is a significant problem in the perioperative period, increasing patient morbidity, mortality, and health care costs. It is also considered the most preventable of the major postoperative complications. Despite widespread adoption of prophylaxis guidelines, it appears that morbidity from the disease has not substantially changed within the past 2 decades. It is becoming clear that current prophylaxis efforts are not sufficient. Using more potent anticoagulants may decrease the incidence of VTE, but increase the risk for bleeding and infection...
June 20, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28637352/absence-of-conformational-change-in-complement-factor-3-and-factor-xii-adsorbed-to-acrylate-polymers-is-related-to-a-high-degree-of-polymer-backbone-flexibility
#8
Karin Fromell, Yi Yang, Kristina Nilsson Ekdahl, Bo Nilsson, Mattias Berglin, Hans Elwing
In previous investigations, the authors have examined the adsorption of albumin, immunoglobulin, and fibrinogen to a series of acrylate polymers with different backbone and side-group flexibility. The authors showed that protein adsorption to acrylates with high flexibility, such as poly(lauryl methacrylate) (PLMA), tends to preserve native conformation. In the present study, the authors have continued this work by examining the conformational changes that occur during the binding of complement factor 3 (C3) and coagulation factor XII (FXII)...
June 21, 2017: Biointerphases
https://www.readbyqxmd.com/read/28637314/dna-methylation-signatures-and-coagulation-factors-in-the-peripheral-blood-leucocytes-of-epithelial-ovarian-cancer
#9
Lian Li, Hong Zheng, Yubei Huang, Caiyun Huang, Shuang Zhang, Jing Tian, Pei Li, Anil K Sood, Wei Zhang, Kexin Chen
Solid tumors are increasingly recognized as a systemic disease that is manifested by changes in DNA, RNA, proteins, and metabolites in the blood. Whereas many studies have reported gene mutation events in the circulation, few studies have focused on epigenetic DNA methylation markers. To identify DNA methylation biomarkers in peripheral blood for ovarian cancer, we performed a two-stage epigenome-wide association study. In the discovery stage, we measured genome wide DNA methylation for 485,000 CpG sites in peripheral blood in 24 epithelial ovarian cancer cases and 24 age-matched healthy controls...
June 16, 2017: Carcinogenesis
https://www.readbyqxmd.com/read/28633041/study-of-platelet-activation-hypercoagulable-state-and-the-association-with-pulmonary-hypertension-in-children-with-%C3%AE-thalassemia
#10
REVIEW
Mahmoud Alhosiny Fayed, Hesham El-Sayed Abdel-Hady, Mona Mohammed Hafez, Osama Saad Salama, Youssef Abdelhalim Al-Tonbary
BACKGROUND: The increased survival rate of thalassemic patients has led to unmasking of management related complications which were infrequently encountered. OBJECTIVE: Study the increased coagulation and platelet activation in children with β-thalassemia, to analyze the factors that lead to such hypercoagulable state and to study pulmonary hypertension (PH) in conjunction with platelet activation and hypercoagulable state in children with β-thalassemia. METHODS: 36 Egyptian children with β-thalassemia with a mean age of 9...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28632940/characterization-of-the-i4399m-variant-of-apolipoprotein-a-implications-for-altered-prothrombotic-properties-of-lipoprotein-a
#11
Corey A Scipione, Jackson T McAiney, Daniel J Simard, Zainab A Bazzi, Matthew Gemin, Rocco Romagnuolo, Fraser L Macrae, Robert A Ariëns, Robert A Hegele, Janeen Auld, James W Gauld, Michael B Boffa, Marlys L Koschinsky
BACKGROUND: Lipoprotein(a) (Lp(a)) is a causal risk factor for a variety of cardiovascular diseases. Apolipoprotein(a) (apo(a)), the distinguishing component of Lp(a), is homologous to plasminogen, suggesting that Lp(a) can interfere with the normal fibrinolytic functions of plasminogen. This has implications for the persistence of fibrin clots in the vasculature and hence to atherothrombotic diseases. A single nucleotide polymorphism (rs3798220) in the gene encoding apo(a) has been reported that results in an Ile to Met substitution in the protease-like domain (I4399M variant)...
June 20, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28632925/a-kallikrein-targeting-rna-aptamer-inhibits-the-intrinsic-pathway-of-coagulation-and-reduces-bradykinin-release
#12
K-A Steen-Burrell, J Layzer, B A Sullenger
BACKGROUND: Plasma kallikrein is a serine protease that plays an integral role in many biological processes including coagulation, inflammation, and fibrinolysis. The main function of kallikrein in coagulation is the amplification of activated factor XIIa (FXIIa) generation which ultimately leads to thrombin generation and fibrin clot formation. Kallikrein is generated by FXIIa-mediated cleavage of the zymogen, prekallikrein which is usually complexed with the nonenzymatic cofactor, high-molecular-weight kininogen (HK)...
June 20, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28632435/relative-effects-of-plasma-fibrinogen-concentrate-and-factor-xiii-on-rotem-coagulation-profiles-in-an-in-vitro-model-of-massive-transfusion-in-trauma
#13
David E Schmidt, Märit Halmin, Agneta Wikman, Anders Östlund, Anna Ågren
Massive traumatic haemorrhage is aggravated through the development of trauma-induced coagulopathy, which is managed by plasma transfusion and/or fibrinogen concentrate administration. It is yet unclear whether these treatments are equally potent in ensuring adequate haemostasis, and whether additional factor XIII (FXIII) administration provides further benefits. In this study, we compared ROTEM whole blood coagulation profiles after experimental massive transfusion with different transfusion regimens in an in vitro model of dilution- and transfusion-related coagulopathy...
June 20, 2017: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/28632184/crude-fucoidan-extracts-impair-angiogenesis-in-models-relevant-for-bone-regeneration-and-osteosarcoma-via-reduction-of-vegf-and-sdf-1
#14
Fanlu Wang, Harald Schmidt, Dijana Pavleska, Thees Wermann, Andreas Seekamp, Sabine Fuchs
The marine origin polysaccharide fucoidan combines multiple biological activities. As demonstrated by various studies in vitro and in vivo, fucoidans show anti-viral, anti-tumor, anti-oxidant, anti-inflammatory and anti-coagulant properties, although the detailed molecular action remains to be elucidated. The aim of the present study is to assess the impact of crude fucoidan extracts, on the formation of vascular structures in co-culture models relevant for bone vascularization during bone repair and for vascularization processes in osteosarcoma...
June 20, 2017: Marine Drugs
https://www.readbyqxmd.com/read/28630342/autoantibody-profiling-on-a-plasmonic-nano-gold-chip-for-the-early-detection-of-hypertensive-heart-disease
#15
Xiaoyang Li, Tatiana Kuznetsova, Nicholas Cauwenberghs, Matthew Wheeler, Holden Maecker, Joseph C Wu, Francois Haddad, Hongjie Dai
The role of autoimmunity in cardiovascular (CV) diseases has been increasingly recognized. Autoimmunity is most commonly examined by the levels of circulating autoantibodies in clinical practices. Measurement of autoantibodies remains, however, challenging because of the deficiency of reproducible, sensitive, and standardized assays. The lack of multiplexed assays also limits the potential to identify a CV-specific autoantibody profile. To overcome these challenges, we developed a nanotechnology-based plasmonic gold chip for autoantibody profiling...
June 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28628948/platelet-hyperaggregability-is-associated-with-decreased-adamts13-activity-and-enhanced-endotoxemia-in-patients-with-acute-cholangitis
#16
Hiroaki Takaya, Hideto Kawaratani, Takuya Kubo, Kenichiro Seki, Yasuhiko Sawada, Kosuke Kaji, Yasushi Okura, Kosuke Takeda, Mitsuteru Kitade, Kei Moriya, Tadashi Namisaki, Akira Mitoro, Masanori Matsumoto, Hiroshi Fukui, Hitoshi Yoshiji
AIM: Insufficient ADAMTS13 activity (ADAMTS13:AC) leads to increased levels of unusually large von Willebrand factor (VWF) multimers and causes microcirculatory disturbance and multiple organ failure (MOF). Endotoxin (Et) triggers the activation of coagulation and cytokine cascades, leading to MOF in severe inflammatory response syndrome (SIRS). Here, we investigated the potential role of endotoxemia-related ADAMTS13 in acute cholangitis. METHODS: Twenty-four patients with acute cholangitis, including 7 with severe acute cholangitis, were recruited in this study...
June 19, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28628602/procoagulant-activity-of-human-mesenchymal-stem-cells
#17
Barbara A Christy, Maryanne C Herzig, Robbie K Montgomery, Christopher Delavan, James A Bynum, Kristin M Reddoch, Andrew P Cap
BACKGROUND: Allogeneic mesenchymal stem cells (MSCs) show great potential for the treatment of military and civilian trauma based on their reduced immunogenicity and ability to modulate inflammation and immune function in the recipient. Although generally considered to be safe, MSCs express tissue factor (TF), a potent activator of coagulation. In the current study, we evaluated multiple MSC populations for tissue factor expression and procoagulant activity to characterize safety considerations for systemic use of MSCs in trauma patients who may have altered coagulation homeostasis...
July 2017: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/28628220/importance-of-endothelial-dysfunction-biomarkers-in-patients-with-crimean-congo-hemorrhagic-fever
#18
Mustafa Arslan, Gürdal Yilmaz, Ahmet Mentese, Hülya Yilmaz, S Caner Karahan, Iftihar Koksal
BACKGROUND: The pathogenesis of the Crimean-Congo hemorrhagic fever (CCHF) and the cause of the hemorrhage are not yet fully understood. However, the endothelium plays a key role in the pathogenesis. The purpose of this study was to investigate endothelial dysfunction markers [asymmetrical dimethyl arginine(ADMA), endothelin 1(ET-1), thrombomodulin(TM), von Willebrand factor(vWf) and intercellular adhesion molecule(ICAM-1)] in serum in patients with CCHF and their associations with hemorrhage...
June 19, 2017: Journal of Medical Virology
https://www.readbyqxmd.com/read/28625825/red-blood-cell-transfusion-in-surgical-cancer-patients-targets-risks-mechanistic-understanding-and-further-therapeutic-opportunities
#19
REVIEW
Vassilis L Tzounakas, Jerard Seghatchian, Elissavet Grouzi, Styliani Kokoris, Marianna H Antonelou
Anemia is present in more than half of cancer patients and appears to be an independent prognostic factor of short- and long-term adverse outcomes. It increases in the advanced period of cancer and perioperatively, in patients with solid tumors who undergo surgery. As a result, allogeneic red blood cell (RBC) transfusion is an indispensable treatment in cancer. However, its safety remains controversial, based on several laboratory and clinical data reporting a linkage with increased risk for cancer recurrence, infection and cancer-related mortality...
May 26, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28625233/-elevation-of-red-cell-distribution-width-during-hospitalization-predicts-mortality-in-patients-with-sepsis
#20
Yan Gong, Xianming Long, Jun Jin, Xinjing Yang, Jianhong Fu, Fang Huang, Jian Huang, Qiang Guo, Jun Wang
OBJECTIVE: To investigate the association of red cell distribution width (RDW) with prognosis in patients with sepsis. METHODS: Patients with sepsis admitted to intensive care unit (ICU) of the First Hospital of Soochow University from January 2011 to December 2016 were enrolled. All clinical data were collected for participants, which mainly included basic data, main underlying disease, site of infection, acute physiology and chronic health evaluation II(APACHE II) score, blood routine test, biochemical test, blood gas analysis, coagulation index, procalcitonin (PCT), hospitalization days, and 28-day and 90-day mortality...
June 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
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