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Von Willebrand Factor

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https://www.readbyqxmd.com/read/28453902/lighting-up-kinase-action-in-platelets
#1
Albert Smolenski
Platelet functions are highly regulated by signaling networks involving at least 300 protein kinases [2]. Platelets express members of the mitogen-activated protein kinase (MAPK) family including p42 ERK2 and, at lower concentrations, p44 ERK1. Both ERK isoforms are activated by agonists like von Willebrand factor, collagen, thrombin, ADP and thromboxane A2 [3-7]. ERK activation depends on MAPK kinase (MKK-1 or MEK) and involves Gq, Gi, phospholipase C and Src family kinase signaling [5, 8]. Active ERK has been shown to stimulate thromboxane A2 production [5] and to contribute to platelet aggregation [4, 9]...
April 28, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28453889/discrepant-platelet-and-plasma-von-willebrand-factor-in-von-willebrand-disease-patients-with-p-pro2808leufs-24
#2
Mackenzie L Bowman, Fred G Pluthero, Angie Tuttle, Lara Casey, Ling Li, Hilary Christensen, K Sue Robinson, David Lillicrap, Walter H A Kahr, Paula James
BACKGROUND: A novel variant, p.Pro2808Leufs*24, in the von Willebrand factor (VWF) gene was previously identified in the Canadian von Willebrand disease (VWD) patient population. Clinical observations of type 3 VWD patients with this variant indicate a milder bleeding phenotype compared to other type 3 patients. OBJECTIVE: To assess the effect of the Pro2808Leufs*24 variant on the molecular pathogenesis of VWD and correlate with the phenotype observed in patients...
April 28, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28449313/neurovascular-unit-alteration-in-somatosensory-cortex-and-enhancement-of-thermal-nociception-induced-by-amphetamine-involves-central-at1-receptor-activation
#3
Victoria Belén Occhieppo, Natalia Andrea Marchese, Iara Diamela Rodríguez, Osvaldo Martín Basmadjian, Gustavo Baiardi, Claudia Bregonzio
The use of psychostimulants, such as amphetamine (Amph), is associated with inflammatory processes, involving glia and vasculature alterations. Brain Angiotensin II (Ang II), through AT1 -receptors (AT1 -R), modulates neurotransmission and plays a crucial role in inflammatory responses in brain vasculature and glia. Our aim for the present work was to evaluate the role of AT1 -R in long-term alterations induced by repeated exposure to Amph. Astrocyte reactivity, neuronal survival and brain microvascular network were analyzed at the somatosensory cortex...
April 27, 2017: European Journal of Neuroscience
https://www.readbyqxmd.com/read/28447419/controversies-in-the-diagnosis-of-type-1-von-willebrand-disease
#4
REVIEW
M L Bowman, P D James
von Willebrand disease (VWD) is the most common bleeding disorder. Type 1 VWD represents the majority of cases and results from a partial quantitative deficiency of von Willebrand factor (VWF). The diagnosis of Type 1 VWD presents many challenges, despite there being three established diagnostic criteria: a personal history of mucocutaneous bleeding, a family history and low VWF levels. These criteria do not always coexist, and there is great overlap of clinical phenotypes and laboratory parameters between healthy individuals and those with VWD...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28447417/diagnostic-approach-to-microangiopathic-hemolytic-disorders
#5
REVIEW
K Kottke-Marchant
Thrombotic micro-angiopathies (TMA) are a group of related disorders that are characterized by thrombosis of the microvasculature and associated organ dysfunction, and encompass congenital, acquired, and infectious etiologies. A hall mark of TMAs is the fragmentation of erythrocytes by the microvascular thrombi, resulting in a hemolytic anemia. There are several distinct pathophysiologies leading to microangiopathic hemolysis, ranging from decreased degradation of von Willebrand factor as seen in thrombotic thrombocytopenic purpura (TTP) to endothelial damage facilitated by Escherichia coli shiga toxin or complement dysregulation, seen in shiga toxin-related hemolytic-uremic syndrome (Stx-HUS) and complement-mediated TMA (also called atypical hemolytic-uremic syndrome), respectively...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28446313/-effect-of-agkistrodon-acutus-venom-pca-on-huvec-activity
#6
Jin-Feng Sang, Gen-Bao Zhang, Shu-Yan Zhou, Na Ji
OBJECTIVE: To investigate the effects of Agkistrodon acutus venom protein C activator(PCA) on ultrastructure of human umbilical vein endothelial cells(HUVEC), and the levels of tissue factor(TF), vascular von Willebrand factor (vWF) and endothelin-1 secreted by HUVEC and to clarify the anti-thrombotic mechanism of PCA. METHODS: The experiments were divided into control group(DMEM), LPS group (LPS 0.1 µg/ml), PCA group(PCA 1 µg/ml) and PCA+LPS group (1 µg/ml PCA+ 0...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28446234/retinal-vessel-diameters-and-reactivity-in-diabetes-mellitus-and-or-cardiovascular-disease
#7
R Heitmar, G Y H Lip, R E Ryder, A D Blann
BACKGROUND: Retinal vessel calibre and vascular dilation/constriction in response to flicker light provocation may provide a measure distinguishing patients suffering from diabetes mellitus and/or cardiovascular disease. METHODS: One hundred and sixteen age and sex matched patients with diabetes mellitus (DM), cardiovascular disease (CVD) and both DM and CVD (DM + CVD) underwent systemic and intraocular pressure measurements. Retinal vessel calibres were assessed using a validated computer-based program to compute central retinal artery and vein equivalents (CRVE) from monochromatic retinal images...
April 26, 2017: Cardiovascular Diabetology
https://www.readbyqxmd.com/read/28442771/double-stranded-dna-induces-a-prothrombotic-phenotype-in-the-vascular-endothelium
#8
Erik Gaitzsch, Thomas Czermak, Andrea Ribeiro, Yvonn Heun, Monica Bohmer, Monika Merkle, Hanna Mannell, Christian Schulz, Markus Wörnle, Joachim Pircher
Double-stranded DNA (dsDNA) constitutes a potent activator of innate immunity, given its ability to bind intracellular pattern recognition receptors during viral infections or sterile tissue damage. While effects of dsDNA in immune cells have been extensively studied, dsDNA signalling and its pathophysiological implications in non-immune cells, such as the vascular endothelium, remain poorly understood. The aim of this study was to characterize prothrombotic effects of dsDNA in vascular endothelial cells. Transfection of cultured human endothelial cells with the synthetic dsDNA poly(dA:dT) induced upregulation of the prothrombotic molecules tissue factor and PAI-1, resulting in accelerated blood clotting in vitro, which was partly dependent on RIG-I signalling...
April 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28441050/comparison-of-platelet-function-and-viscoelastic-test-results-between-healthy-dogs-and-dogs-with-naturally-occurring-chronic-kidney-disease
#9
Alicia Dudley, Julie K Byron, Mary Jo Burkhard, Emma Warry, Julien Guillaumin
OBJECTIVE To compare platelet function and viscoelastic test results between healthy dogs and dogs with chronic kidney disease (CKD) to assess whether dogs with CKD have platelet dysfunction and altered blood coagulation. ANIMALS 10 healthy control dogs and 11 dogs with naturally occurring CKD. PROCEDURES Blood and urine were collected once from each dog for a CBC, serum biochemical analysis, urinalysis, and determination of the urine protein-to-creatinine ratio, prothrombin time, activated partial thromboplastin time, plasma fibrinogen concentration, and antithrombin activity...
May 2017: American Journal of Veterinary Research
https://www.readbyqxmd.com/read/28441034/thrombus-formation-at-high-shear-rate
#10
Lauren D C Casa, David N Ku
The final common pathway in myocardial infarction and ischemic stroke is occlusion of blood flow from a thrombus forming under high shear rates in arteries. A high-shear thrombus forms rapidly and is distinct from the slow formation of coagulation that occurs in stagnant blood. Thrombosis at high shear rates depends primarily on the long protein von Willebrand factor (vWF) and platelets, with hemodynamics playing an important role in each stage of thrombus formation, including vWF binding, platelet adhesion, platelet activation, and rapid thrombus growth...
April 24, 2017: Annual Review of Biomedical Engineering
https://www.readbyqxmd.com/read/28437192/phototoxicity-of-vascular-endothelial-cells-caused-by-contact-with-talaporfin-sodium-for-15-120-min-in-vitro-and-in-vivo-studies
#11
Risa Hamada, Emiyu Ogawa, Tsunenori Arai
OBJECTIVE: To reveal the mechanism of vascular patency in the myocardium after photosensitization immediately after talaporfin sodium (TS) injection in a canine model, we investigated acute injury to vascular endothelial cells (VECs) in vitro and in vivo. BACKGROUND DATA: There are many reports of vascular shutdown within the target region in photodynamic therapy with TS. Vascular patency within healthy canine myocardium in which a photosensitization reaction starts immediately after injection of TS has been reported...
February 22, 2017: Photomedicine and Laser Surgery
https://www.readbyqxmd.com/read/28436749/differential-diagnosis-of-neonatal-alloimmune-thrombocytopenia-type-2b-von-willebrand-disease
#12
Mathilde Penel-Page, Sandrine Meunier, Mathilde Fretigny, Sandra Le Quellec, Pierre Boisseau, Christine Vinciguerra, Catherine Ternisien, Lucia Rugeri
At birth, severe thrombocytopenia without context of infection should mainly suggest neonatal alloimmune thrombocytopenia (NAIT), especially in case of a platelet count below 20 GL(-1). We report two cases of severe neonatal thrombocytopenia, first suspected as being NAIT. Both had a platelet count below 20 GL(-1) with platelet clumps. The absence of alloantibodies and failure of platelet transfusion and intravenous immunoglobulins to improve the platelet count led to question the diagnosis and to evoke inherited bleeding disorders...
April 24, 2017: Platelets
https://www.readbyqxmd.com/read/28434015/low-lymphatic-vessel-density-associates-with-chronic-rhinosinusitis-with-nasal-polyps
#13
A Luukkainen, M Seppälä, J Renkonen, R Renkonen, J Hagström, H Huhtala, M Rautiainen, J Myller, T Paavonen, A Ranta, T Torkkeli, S Toppila-Salmi
OBJECTIVES: Chronic rhinosinusitis with and without nasal polyps (CRSwNP and CRSsNP) and antrochoanal polyps (ACP) are different upper airway inflammation phenotypes with different pathomechanisms. In order to understand the development of tissue edema, the present study aimed to evaluate lymphatic vessel density in CRSsNP, CRSwNP and ACP. MATERIALS AND METHODS: 120 retrospective nasal and maxillary sinus specimens were stained immunohistochemically with a von Willebrand factor polyclonal antibody recognizing vascular and lymphatic endothelium, and with a podoplanin monoclonal antibody recognizing lymphatic endothelium...
April 23, 2017: Rhinology
https://www.readbyqxmd.com/read/28432157/effect-of-interleukin-6-inhibition-on-coronary-microvascular-and-endothelial-function-in-myocardial-infarction
#14
Espen Holte, Ola Kleveland, Thor Ueland, Gabor Kunszt, Marte Bratlie, Kaspar Broch, Annika E Michelsen, Bjørn Bendz, Brage H Amundsen, Svend Aakhus, Jan Kristian Damås, Lars Gullestad, Pål Aukrust, Rune Wiseth
OBJECTIVE: Interleukin-6 (IL-6) is a driver of inflammation and associated endothelial cell activation in acute coronary syndromes. We evaluated the effect of the IL-6 receptor antagonist tocilizumab on coronary microvascular function and endothelial dysfunction measured by coronary flow reserve (CFR) and markers of endothelial cell activation in patients with non-ST-elevation myocardial infarction (NSTEMI). METHODS: This substudy was part of a two-centre, double-blind, randomised, placebo-controlled trial evaluating the effect of a single dose of tocilizumab in NSTEMI...
April 21, 2017: Heart: Official Journal of the British Cardiac Society
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/28428218/gain-of-function-mutation-in-filamin-a-potentiates-platelet-integrin-%C3%AE-iib%C3%AE-3-activation
#16
Eliane Berrou, Frédéric Adam, Marilyne Lebret, Virginie Planche, Patricia Fergelot, Odile Issertial, Isabelle Coupry, Jean-Claude Bordet, Paquita Nurden, Dominique Bonneau, Estelle Colin, Cyril Goizet, Jean-Philippe Rosa, Marijke Bryckaert
OBJECTIVE: Dominant mutations of the X-linked filamin A (FLNA) gene are responsible for filaminopathies A, which are rare disorders including brain periventricular nodular heterotopia, congenital intestinal pseudo-obstruction, cardiac valves or skeleton malformations, and often macrothrombocytopenia. APPROACH AND RESULTS: We studied a male patient with periventricular nodular heterotopia and congenital intestinal pseudo-obstruction, his unique X-linked FLNA allele carrying a stop codon mutation resulting in a 100-amino acid-long FLNa C-terminal extension (NP_001447...
April 20, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28420732/modulation-of-tmem16a-channel-activity-by-the-von-willebrand-factor-type-a-vwa-domain-of-the-calcium-activated-chloride-channel-regulator-1-clca1
#17
Monica Sala-Rabanal, Zeynep Yurtsever, Kayla N Berry, Colin G Nichols, Tom J Brett
Calcium-activated chloride channels (CaCCs) are key players in transepithelial ion transport and fluid secretion, smooth muscle constriction, neuronal excitability, and cell proliferation. The CaCC regulator 1 (CLCA1) modulates the activity of the CaCC TMEM16A/Anoctamin 1 (ANO1) by directly engaging the channel at the cell surface, but the exact mechanism is unknown. Here, we demonstrate that the von Willebrand factor type A (VWA) domain within the cleaved CLCA1 N-terminal fragment is necessary and sufficient for this interaction...
April 18, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#18
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28415932/the-effects-of-corrective-surgery-on-endothelial-biomarkers-and-anthropometric-data-in-children-with-congenital-heart-disease
#19
Hung-Tao Chung, Yu-Sheng Chang, Sui-Ling Liao, Shen-Hao Lai
Objective To investigate the influence of surgical correction on biomarkers of endothelial dysfunction in children with congenital heart disease and to evaluate anthropometric data. Methods Children with pulmonary hypertension (PH) or Tetralogy of Fallot (TOF) who were scheduled for corrective surgery were enrolled in this prospective study. Age-matched healthy children were included as controls. Demographic, haemodynamic and cardiac ultrasonography data were collected. Blood samples were taken pre-surgery, 24-48 hours post-surgery and again 3-6 months later...
April 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28414126/characterization-of-the-novel-protein-kiaa0564-von-willebrand-domain-containing-protein-8
#20
Moulun Luo, April E Mengos, Wuqiong Ma, Jean Finlayson, Rocio Zapata Bustos, Yuan Xiao Zhu, Chang-Xin Shi, Tianna M Stubblefield, Wayne T Willis, Lawrence J Mandarino
The VWA8 gene was first identified by the Kazusa cDNA project and named KIAA0564. Based on the observation, by similarity, that the protein encoded by KIAA0564 contains a Von Willebrand Factor 8 domain, KIAA0564 was named Von Willebrand Domain-containing Protein 8 (VWA8). The function of VWA8 protein is almost unknown. The purpose of this study was to characterize the tissue distribution, cellular location, and function of VWA8. In mice VWA8 protein was mostly distributed in liver, kidney, heart, pancreas and skeletal muscle, and is present as a long isoform and a shorter splice variant (VWA8a and VWA8b)...
April 13, 2017: Biochemical and Biophysical Research Communications
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