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https://www.readbyqxmd.com/read/29040872/highly-elevated-plasma-level-of-von-willebrand-factor-accelerates-the-formation-of-platelet-thrombus-under-high-shear-stress-in-plasma-with-deficient-adamts13-activity
#1
Hideo Yagi, Naoko Yamaguchi, Yasuaki Shida, Mitsuhiko Sugimoto, Kazuo Tubaki, Yoshihiro Fujimura, Masanori Matsumoto
Upshaw-Schulman syndrome (USS) is a thrombo-hemorrhagic disease caused by congenital deficiency of ADAMTS13 due to ADAMTS13 gene mutations. USS is characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond dramatically to infusions of fresh frozen plasma. There are two phenotypic expressions of USS: one is the early-onset type and the other, the late-onset type, is asymptomatic during childhood with the first bout of thrombotic thrombocytopenic purpura (TTP) developing after adolescence or during adulthood...
October 10, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28993756/trench-foot-or-non-freezing-cold-injury-as-a-painful-vaso-neuropathy-clinical-and-skin-biopsy-assessments
#2
Praveen Anand, Rosario Privitera, Yiangos Yiangou, Philippe Donatien, Rolfe Birch, Peter Misra
BACKGROUND: Trench foot, or non-freezing cold injury (NFCI), results from cold exposure of sufficient severity and duration above freezing point, with consequent sensory and vascular abnormalities which may persist for years. Based on observations of Trench foot in World War II, the condition was described as a vaso-neuropathy. While some reports have documented nerve damage after extreme cold exposure, sensory nerve fibres and vasculature have not been assessed with recent techniques in NFCI...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28981926/-genetic-and-prenatal-diagnosis-of-a-pedigree-affected-with-type-3-von-willebrand-disease
#3
Haiyan Zhu, Chunyan Ji
OBJECTIVE: To provide genetic and prenatal analysis for a pedigree affected with type 3 von Willebrand disease. METHODS: Next generation sequencing and Sanger sequencing of the VWF gene were carried out for the pedigree. Suscepted pathogenic mutation was verified among other members of the pedigree and 100 healthy controls. Prenatal diagnosis was performed on amniotic cells derived from the fetus. RESULTS: A homozygous mutation c.7287+1G>A of the VWF gene was detected in the patient, which was predicted by bioinformatic analysis as a pathological splice site mutation...
October 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28980147/adamts13-missense-variants-associated-with-defective-activity-and-secretion-of-adamts13-in-a-patient-with-non-cirrhotic-portal-hypertension
#4
Ashish Goel, V Raghupathy, G J Amirtharaj, Aaron Chapla, Aparna Venkatraman, Banumathi Ramakrishna, Anup Ramachandran, Nihal Thomas, K A Balasubramanian, Ian Mackie, Elwyn Elias, Chundamannil E Eapen
BACKGROUND: Non-cirrhotic intrahepatic portal hypertension (NCIPH) is characterized by thrombotic microangiopathy of the portal venous system, low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs-13), and high vWF (von Willebrand factor) levels. This study aimed to screen for ADAMTS13 mutations, focusing on the CUB domain, in these patients. METHODS: Prospectively recruited NCIPH patients and healthy volunteers underwent tests for plasma vWF-ADAMTS13 balance...
October 5, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28930985/visualizing-angiogenesis-by-multiphoton-microscopy-in-vivo-in-genetically-modified-3d-plga-nhap-scaffold-for-calvarial-critical-bone-defect-repair
#5
Jian Li, Holger Jahr, Wei Zheng, Pei-Gen Ren
The reconstruction of critically sized bone defects remains a serious clinical problem because of poor angiogenesis within tissue-engineered scaffolds during repair, which gives rise to a lack of sufficient blood supply and causes necrosis of the new tissues. Rapid vascularization is a vital prerequisite for new tissue survival and integration with existing host tissue. The de novo generation of vasculature in scaffolds is one of the most important steps in making bone regeneration more efficient, allowing repairing tissue to grow into a scaffold...
September 7, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28900857/endovascular-biopsy-in-vivo-cerebral-aneurysm-endothelial-cell-sampling-and-gene-expression-analysis
#6
Daniel L Cooke, David B McCoy, Van V Halbach, Steven W Hetts, Matthew R Amans, Christopher F Dowd, Randall T Higashida, Devon Lawson, Jeffrey Nelson, Chih-Yang Wang, Helen Kim, Zena Werb, Charles McCulloch, Tomoki Hashimoto, Hua Su, Zhengda Sun
There is limited data describing endothelial cell (EC) gene expression between aneurysms and arteries partly because of risks associated with surgical tissue collection. Endovascular biopsy (EB) is a lower risk alternative to conventional surgical methods, though no such efforts have been attempted for aneurysms. We sought (1) to establish the feasibility of EB to isolate viable ECs by fluorescence-activated cell sorting (FACS), (2) to characterize the differences in gene expression by anatomic location and rupture status using single-cell qPCR, and (3) to demonstrate the utility of unsupervised clustering algorithms to identify cell subpopulations...
September 13, 2017: Translational Stroke Research
https://www.readbyqxmd.com/read/28900302/functional-dissection-of-hematopoietic-stem-cell-populations-with-a-stemness-monitoring-system-based-on-ns-gfp-transgene-expression
#7
Mohamed A E Ali, Kyoko Fuse, Yuko Tadokoro, Takayuki Hoshii, Masaya Ueno, Masahiko Kobayashi, Naho Nomura, Ha Thi Vu, Hui Peng, Ahmed M Hegazy, Masayoshi Masuko, Hirohito Sone, Fumio Arai, Atsushi Tajima, Atsushi Hirao
Hematopoietic stem cells (HSCs) in a steady state can be efficiently purified by selecting for a combination of several cell surface markers; however, such markers do not consistently reflect HSC activity. In this study, we successfully enriched HSCs with a unique stemness-monitoring system using a transgenic mouse in which green florescence protein (GFP) is driven by the promoter/enhancer region of the nucleostemin (NS) gene. We found that the phenotypically defined long-term (LT)-HSC population exhibited the highest level of NS-GFP intensity, whereas NS-GFP intensity was strongly downregulated during differentiation in vitro and in vivo...
September 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28899852/identification-of-extant-vertebrate-myxine-glutinosa-vwf-evolutionary-conservation-of-primary-hemostasis
#8
Marianne A Grant, David L Beeler, Katherine C Spokes, Junmei Chen, Harita Dharaneeswaran, Tracey E Sciuto, Ann M Dvorak, Gianluca Interlandi, José A Lopez, William C Aird
Hemostasis in vertebrates involves both a cellular and protein component. Previous studies in jawless vertebrates (cyclostomes) suggest that the protein response, which involves thrombin-catalyzed conversion of a soluble plasma protein, fibrinogen, into a polymeric fibrin clot, is conserved in all vertebrates. However, similar data are lacking for the cellular response, which in gnathostomes, is regulated by von Willebrand factor (VWF), a glycoprotein that mediates the adhesion of platelets to the subendothelial matrix of injured blood vessels...
September 12, 2017: Blood
https://www.readbyqxmd.com/read/28868583/von-willebrand-factor-protects-against-acute-ccl4-induced-hepatotoxicity-through-phospho-p38-mapk-signaling-pathway-inhibition
#9
Hai-Jian Sun, Jian Chen, Hao Zhang, Bing Ni, Jennifer C van Velkinburgh, Yao Liu, Yu-Zhang Wu, Xia Yang
The blood glycoprotein von Willebrand factor (vWF) is involved in coagulopathy and inflammation; however, its role in the pathogenesis of acute liver failure, as suggested by its higher expression levels in such patients, remains unknown. In this study, vWF-knockout (KO) mice showed more severe carbon tetrachloride (CCl4)-induced liver injury than wild-type mice. Patients with acute liver injury also showed elevated vWF protein activity and expression in liver tissues, as compared to healthy individuals. Using the mouse model and cultured human umbilical vein endothelial cells (HUVECs), CCl4 was found to directly increase vWF protein expression through interaction with the highly expressed vWF receptor, GPIbα...
September 3, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28791655/current-and-emerging-options-for-the-management-of-inherited-von-willebrand-disease
#10
REVIEW
Jessica M Heijdra, Marjon H Cnossen, Frank W G Leebeek
Von Willebrand disease (VWD) is the most common inherited bleeding disorder with an estimated prevalence of ~1% and clinically relevant bleeding symptoms in approximately 1:10,000 individuals. VWD is caused by a deficiency and/or defect of von Willebrand factor (VWF). The most common symptoms are mucocutaneous bleeding, hematomas, and bleeding after trauma or surgery. For decades, treatment to prevent or treat bleeding has consisted of desmopressin in milder cases and of replacement therapy with plasma-derived concentrates containing VWF and Factor VIII (FVIII) in more severe cases...
August 8, 2017: Drugs
https://www.readbyqxmd.com/read/28714856/use-of-human-aortic-extracellular-matrix-as-a-scaffold-for-construction-of-patient-specific-tissue-engineered-vascular-patch
#11
Liping Gao, Mingjun Du, Jingjing Lv, Sebastian Schmull, Ritai Huang, Jun Li
Abstract Synthetic or biologic materials are usually used to repair vascular malformation in congenital heart defects; however, non-autologous materials show both mismatch compliance and antigenicity, as well as a lack of recellularization on its surface. Here, we constructed a tissue-engineered vascular patch (TEVP) using decellularized extracellular matrix (ECM) scaffold obtained from excised human aorta during surgery, which was seeded with patient-derived bone marrow CD34-positive (CD34+) progenitor cells...
July 17, 2017: Biomedical Materials
https://www.readbyqxmd.com/read/28702926/the-stability-evaluation-of-mesenchymal-stem-cells-differentiation-toward-endothelial-cells-by-chemical-and-mechanical-stimulation
#12
Elham Hasanzadeh, Ghassem Amoabediny, Nooshin Haghighipour, Nasim Gholami, Javad Mohammadnejad, Shahrokh Shojaei, Nasim Salehi-Nik
Adipose-derived mesenchymal stem cells (ADSCs) are adult multipotent cells able to differentiate into several cell lineages. Vascular endothelial growth factor (VEGF) and the shear stress associated with blood flow are considered as the most important chemical and mechanical cues that play major roles in endothelial differentiation. However, the stability of endothelial-specific gene expression has not been completely addressed yet. ADSCs in passage 3 were cultured inside the tubular silicon tubes and then exposed to VEGF or shear stress produced in a perfusion bioreactor...
October 2017: In Vitro Cellular & Developmental Biology. Animal
https://www.readbyqxmd.com/read/28693615/genome-analysis-of-clostridium-perfringens-isolates-from-healthy-and-necrotic-enteritis-infected-chickens-and-turkeys
#13
Troels Ronco, Marc Stegger, Kim Lee Ng, Berit Lilje, Ulrike Lyhs, Paal Skytt Andersen, Karl Pedersen
OBJECTIVE: Clostridium perfringens causes gastrointestinal diseases in both humans and domestic animals. Type A strains expressing the NetB toxin are the main cause of necrotic enteritis (NE) in chickens, which has remarkable impact on animal welfare and production economy in the international poultry industry. Three pathogenicity loci NELoc-1, -2 and -3 and a collagen adhesion gene cnaA have been found to be associated with NE in chickens, whereas the presence of these has not been investigated in diseased turkeys...
July 11, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28686347/epo-modified-mscs-can-inhibit-asthmatic-airway-remodeling-in-an-animal-model
#14
Xin-Peng Han, Fang-Qi Zhang, Xiang-Shu Tan, Liang Liu, Wen-Xian Ma, Hai-Feng Ou-Yang, Chang-Gui Wu
There was no effective measures can be obtained at present to reverse or prevent airway remodeling. We investigated the therapeutic effect of Erythropoietin (EPO) gene modified mesenchymal stem cells (MSCs) on asthmatic airway remodeling and the possible underlied molecular mechanisms. EPO gene was transfected into MSCs via lentivirus vector. The transfected cells (EPO-MSCs) were identified by flow cytometry and the EPO secreting function was detected by PCR and Western blot. MSCs or EPO-MSCs were administrated to albumin (OVA)-induced chronic asthmatic mouse model via tail veins...
July 7, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28662310/thrombotic-thrombocytopenic-purpura-pathogenesis-diagnosis-and-potential-novel-therapeutics
#15
REVIEW
M Saha, J K McDaniel, X L Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal clinical syndrome, is primarily caused by autoantibodies against the von Willebrand factor (VWF)-cleaving metalloprotease ADAMTS-13. In general, severe deficiency of plasma ADAMTS-13 activity (< 10 IU dL(-1) ) with or without detectable inhibitory autoantibodies against ADAMTS-13 supports the diagnosis of TTP. A patient usually presents with thrombocytopenia and microangiopathic hemolytic anemia (i.e. schistocytes, elevated serum lactate dehydrogenase, decreased hemoglobin and haptoglobin) without other known etiologies that cause thrombotic microangiopathy (TMA)...
October 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28544236/clinical-and-laboratory-phenotype-variability-in-type-2m-von-willebrand-disease
#16
A L Doruelo, S L Haberichter, P A Christopherson, L N Boggio, S Gupta, S R Lentz, A D Shapiro, R R Montgomery, V H Flood
Essentials The pathophysiology of type 2M von Willebrand disease (VWD) is poorly understood. Sequence variations in type 2M VWD subjects were characterized. A high degree of clinical and laboratory variability exists within type 2M VWD variants. Some type 2M variants may share features of type 2A VWD. SUMMARY: Background von Willebrand factor (VWF) is a multimeric coagulation factor that tethers platelets to injured subendothelium. Type 2M von Willebrand disease (VWD) is characterized by a qualitative defect in VWF with preserved multimer distribution...
August 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28527913/von-willebrand-factor-deficiency-reduces-liver-fibrosis-in-mice
#17
Nikita Joshi, Anna K Kopec, Jessica L Ray, Holly Cline-Fedewa, Dafna J Groeneveld, Ton Lisman, James P Luyendyk
Liver diseases are associated with complex changes in the hemostatic system and elevated levels of the platelet-adhesive protein Von Willebrand factor (VWF) are reported in patients with acute and chronic liver damage. Although elevated levels of VWF are associated with fibrosis in the general population, the role of VWF in acute and chronic liver injury has not been examined in depth in experimental settings. We tested the hypothesis that VWF deficiency inhibits experimental liver injury and fibrosis. Wild-type (WT) and VWF-deficient mice were challenged with carbon tetrachloride (CCl4) and the impact of VWF deficiency on acute liver injury and chronic liver fibrosis was determined...
August 1, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28503498/evaluation-of-energy-balance-on-human-telomerase-reverse-transcriptase-htert-alternative-splicing-by-semi-quantitative-rt-pcr-in-human-umbilical-vein-endothelial-cells
#18
Mohaddeseh Behjati, Mohammad Hashemi, Mohammad Kazemi, Mansoor Salehi, Shaghayegh Haghjooy Javanmard
BACKGROUND: Decreased high-energy phosphate level is involved in endothelial cell injury and dysfunction. Reduced telomerase activity in endothelial cells in parallel with reduced energy levels might be due to altered direction of alternative splicing machine as a complication of depleted energy during the process of atherosclerosis. MATERIALS AND METHODS: Isolated human umbilical vein endothelial cells (HUVECs) were treated for 24 hours by oligomycine (OM) and 2-deoxy glucose (2-DG)...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28495826/genetic-drivers-of-von-willebrand-factor-levels-in-an-ischemic-stroke-population-and-association-with-risk-for-recurrent-stroke
#19
RANDOMIZED CONTROLLED TRIAL
Stephen R Williams, Fang-Chi Hsu, Keith L Keene, Wei-Min Chen, Godfrey Dzhivhuho, Joe L Rowles, Andrew M Southerland, Karen L Furie, Stephen S Rich, Bradford B Worrall, Michèle M Sale
BACKGROUND AND PURPOSE: von Willebrand factor (vWF) plays an important role in thrombus formation during cerebrovascular damage. We sought to investigate the potential role of circulating vWF in recurrent cerebrovascular events and identify genetic contributors to variation in vWF level in an ischemic stroke population. METHODS: We analyzed the effect of circulating vWF on risk of recurrent stroke using survival models in the VISP trial (Vitamin Intervention for Stroke Prevention) and the use of vWF in reclassification over traditional factors...
June 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28456355/potential-mechanisms-of-vascular-thrombosis-after-microwave-ablation-in-an%C3%A2-in%C3%A2-vivo-liver
#20
Jason Chiang, Kwang Nickel, Randall J Kimple, Christopher L Brace
PURPOSE: To evaluate potential biologic and thermal mechanisms of the observed differences in thrombosis rates between hepatic vessels during microwave (MW) ablation procedures. MATERIALS AND METHODS: MW ablation antennae were placed in single liver lobes of 2 in vivo porcine liver models (n = 3 in each animal; N = 6 total) in the proximity of a large (> 5 mm) portal vein (PV) and hepatic veins (HVs). Each ablation was performed with 100 W for 5 minutes. Conventional ultrasound imaging and intravascular temperature probes were used to evaluate vessel patency and temperature changes during the ablation procedure...
July 2017: Journal of Vascular and Interventional Radiology: JVIR
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