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Modifier and VWF

A S Faiz, A Kaveney, S Guo, S Murphy, C S Philipp
INTRODUCTION: Family members of Von Willebrand disease (VWD) patients may have low levels of VWF without major bleeding episodes and often remain undiagnosed. AIM: The purpose of this study was to assess the utility of a modified Screening Tool in identifying previously untested reproductive age female family members of VWD patients for haemostatic evaluation. METHODS: Ninety-four reproductive age women including 41 previously untested family members of VWD patients, 26 previously diagnosed VWD patients and 27 healthy controls were administered a modified Screening Tool and had blood drawn for CBC, ferritin, and VWF testing...
February 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
Wenbin Dong, Yunping Miao, Aiying Chen, Min Cheng, Xiaodi Ye, Fahuan Song, Gaoli Zheng
Glucagon-like peptide 1 receptor (GLP-1R) agonists administered before or immediately after induction of experimental stroke have been shown to provide acute neuroprotection. Here, we determined whether delayed treatment with a GLP-1R agonist could improve metabolic and functional recovery after stroke. Rats were subjected to middle cerebral artery occlusion (MCAO) and given the well-established GLP-1R agonist liraglutide (50, 100, or 200μg/kg) or normal saline (NS) daily for 4 weeks, starting 1 day after MCAO...
January 22, 2017: Neuroscience Letters
Maria Isabel Castellanos, Jordi Guillem-Marti, Carlos Mas-Moruno, Maribel Díaz-Ricart, Ginés Escolar, Maria Pau Ginebra, Francisco Javier Gil, Marta Pegueroles, Jose María Manero
Immobilization of bioactive peptide sequences on CoCr surfaces is an effective route to improve endothelialization, which is of great interest for cardiovascular stents. In this work, we explored the effect of physical and covalent immoblization of RGDS, YIGSR and their equimolar combination peptides on endothelial cells (EC) and smooth muscle cell (SMC) adhesion and on thrombogenicity. We extensively investigated using RT-qPCR, the expression by ECs cultured on functionalised CoCr surfaces of different genes...
December 21, 2016: Journal of Biomedical Materials Research. Part A
Patrycja Sosińska, Ewa Baum, Beata Maćkowiak, Magdalena Maj, Katarzyna Sumińska-Jasińska, Ryszard Staniszewski, Andrzej Bręborowicz
BACKGROUND/AIMS: Dysfunction of the arterial endothelial cells promotes the progression of atherosclerosis. We studied how exposure of human arterial endothelial cells to atherosclerotic serum from patients with peripheral artery disease changes the secretory activity of these cells, and whether that reaction is modified by sulodexide. METHODS: Endothelial cells in in vitro culture were exposed to standard culture medium ± 100pg/mL Interleukin-1(IL-1) or to medium supplemented with 20% atherosclerotic serum...
2016: Cellular Physiology and Biochemistry
Binzhi Tang, Dejian Wang, Maojun Li, Qing Wu, Qian Yang, Wei Shi, Changhui Chen
BACKGROUND: Hypoxia/ischemia (HI) brain injury is a common central nervous system insult in newborns. Studies have demonstrated bioactivity of ginsenoside Rg1 in increasing neural viability and promoting angiogenesis. However, there are few reports on roles of Rg1 in brain repair of neonatal HI, and the mechanisms involved are unclear. METHODS: a neonatal HI model was established by a modified Rice-Vannucci model (RVM) and pups received ginsenoside Rg1 or monosialotetrahexosyl ganglioside (GM1) treatment...
January 2017: Pediatric Research
Francesco Ferraro, Silva Mafalda Lopes da, William Grimes, Hwee Kuan Lee, Robin Ketteler, Janos Kriston-Vizi, Daniel F Cutler
Changes in the size of cellular organelles are often linked to modifications in their function. Endothelial cells store von Willebrand Factor (vWF), a glycoprotein essential to haemostasis in Weibel-Palade bodies (WPBs), cigar-shaped secretory granules that are generated in a wide range of sizes. We recently showed that forcing changes in the size of WPBs modifies the activity of this cargo. We now find that endothelial cells treated with statins produce shorter WPBs and that the vWF they release at exocytosis displays a reduced capability to recruit platelets to the endothelial cell surface...
2016: Scientific Reports
Junjie Yang, Xiaofei Zhang, Zhenao Zhao, Xizhe Li, Xu Wang, Ming Chen, Bo Song, Masaaki Ii, Zhenya Shen
BACKGROUND: Endothelial progenitor cells (EPCs) have shown great potential in angiogenesis either by their differentiation into endothelial cells or by secretion of angiogenic factors. Interferon-inducible protein 204 (Ifi204) has been reported to participate in the regulation of cell growth and differentiation. However, its role in differentiation of EPCs remains unknown. We proposed that Ifi204 could modulate the differentiation and regenerative abilities of EPCs. METHODS: Ifi204-expressing lentivirus and Ifi204 siRNA were introduced into EPCs to overexpress and suppress the expression of Ifi204...
August 11, 2016: Stem Cell Research & Therapy
Laura L Swystun, David Lillicrap
PURPOSE OF REVIEW: In the last nine decades, large advances have been made toward the characterization of the pathogenic basis and clinical management of von Willebrand disease (VWD), the most prevalent inherited bleeding disorder. Pathological variations at the von Willebrand factor (VWF) locus present as a range of both quantitative and qualitative abnormalities that make up the complex clinical spectrum of VWD. This review describes the current understanding of the pathobiological basis of VWD...
September 2016: Current Opinion in Hematology
Cheng-Gang Li, Lin Shen, Yan-Ping Yang, Xiao-Juan Xu, Bo Shuai, Chen Ma
OBJECTIVE: To observe the regulation of Chinese herbal medicine, Modified Qing'e Pill (, MQEP), on the expression of adiponectin, bone morphogenetic protein 2 (BMP2), osteoprotegerin (OPG) and other potentially relevant risk factors in patients with nontraumatic osteonecrosis of the femoral head (ONFH). METHODS: A total of 96 patients with nontraumatic ONFH were unequal randomly divided into treatment group (60 cases) and control group (36 cases). The treatment group were treated with MQEP while the control group were treated with simulated pills...
May 6, 2016: Chinese Journal of Integrative Medicine
Jie Wang, Yue Han, Lu-Ping Hu, De-Pei Wu, Chang-Geng Ruan
OBJECTIVE: To explore the influence of precondintioning on ADAMTS-13 activity and vWF level, and its clinical significance by measuring the alterations of ADAMTS-13 activity and vWF antigen levels before and after preconditioning of hematopoietic stem cell transplantation (HSCT) in the patients. METHOD: A total of 113 patients received HSCT in the First Hospital affiliated to Soochow University were investigated, 20 healthy volunteers were used as the control. The ADAMTS-13 activity and vWF antigen level were measured by FRETS-vWF73 and ELISA respectively...
April 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
D'Andra N Parker, Subia Tasneem, Richard W Farndale, Dominique Bihan, J Evan Sadler, Silvie Sebastian, Philip G de Groot, Catherine P M Hayward
Multimerin 1 (MMRN1) is a massive, homopolymeric protein that is stored in platelets and endothelial cells for activation-induced release. In vitro, MMRN1 binds to the outer surfaces of activated platelets and endothelial cells, the extracellular matrix (including collagen) and von Willebrand factor (VWF) to support platelet adhesive functions. VWF associates with MMRN1 at high shear, not static conditions, suggesting that shear exposes cryptic sites within VWF that support MMRN1 binding. Modified ELISA and surface plasmon resonance were used to study the structural features of VWF that support MMRN1 binding, and determine the affinities for VWF-MMRN1 binding...
July 4, 2016: Thrombosis and Haemostasis
Caroline Ridley, Sara Kirkham, Sally J Williamson, C William Davis, Philip Woodman, David J Thornton
MUC5B is a major polymeric mucin in the airway mucus gel and is an essential component of innate defense of the respiratory epithelium. Knowledge of the synthesis and intracellular processing of MUC5B is incomplete. We investigated the molecular details of MUC5B assembly in primary human bronchial epithelial cells (HBECs) grown at an air-liquid interface (ALI). Electrophoretic and centrifugal separations of intracellular forms of MUC5B probed with antibodies specific for non-O-glycosylated and O-glycosylated forms of the mucin identified three major intracellular populations of MUC5B (non-O-glycosylated monomer and dimer, and O-glycosylated polymers)...
May 15, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
Seung-Chul Kim, Sora An, Hyun-Ki Kim, Beom-Soo Park, Kyu-Heum Na, Byung-Gee Kim
UNLABELLED: This study describes the development and experimental verification of a modified harvest system to enhance Factor VIII (FVIII) yield in an alternating tangential flow (ATF) perfusion culture. The main innovation of the modified harvest system is the use of check and pinch valves, eliminating the need of a peristaltic pump for harvest. The system was applied to perfusion cultures of Chinese hamster ovary cells, which co-express both recombinant human FVIII (rhFVIII) and von Willebrand factor (vWF)...
May 2016: Journal of Bioscience and Bioengineering
Hu Tan, Yaoming Song, Jun Jin, Xiaohui Zhao, Jianfei Chen, Zhexue Qing, Shiyong Yu, Lan Huang
Despite the potential of endothelial progenitor cells (EPCs) to incorporate into sites of vessel injury and differentiate into endothelial cells, thereby contributing to the improvement of endothelial function, reendothelialization in some patients is insufficient for the prevention of abnormal endothelial growth because there is a lack of specific guided signals and low levels of congregation of the EPCs to the injured areas. If some type of molecular tool was able to guide EPCs specifically to the injured vessels, however, then the efficacy of cell implantation would improve...
September 2016: Journal of Drug Targeting
Yi-Wen Chen, Tuan-Ti Hsu, Kan Wang, Ming-You Shie
Develop a fast setting and controllable degrading magnesium-calcium silicate cement (Mg-CS) by sol-gel, and establish a mechanism using Mg ions to stimulate human periodontal ligament cells (hPDLs) are two purposes of this study. We have used the diametral tensile strength measurement to obtain the mechanical strength and stability of Mg-CS cement; in addition, the cement degradation properties is realized by measuring the releasing amount of Si and Mg ions in the simulated body fluid. The other cell characteristics of hPDLs, such as proliferation, differentiation and mineralization were examined while hPDLs were cultured on specimen surfaces...
March 2016: Materials Science & Engineering. C, Materials for Biological Applications
Claudia Tersteeg, Rob Fijnheer, Gerard Pasterkamp, Philip G de Groot, Karen Vanhoorelbeke, Steven de Maat, Coen Maas
Von Willebrand factor (VWF) is one of the most important proteins of the hemostatic system. Its multimeric state is essential for its natural function to guide platelets to sites of injury. ADAMTS13 is the key protease that regulates the multimeric state of VWF. Without ADAMTS13, VWF multimers can grow to pathologically large sizes. This is a risk factor for the life-threatening condition thrombotic thrombocytopenic purpura (TTP). In this condition, VWF-rich thrombi occlude the microvasculature of various tissues...
February 2016: Seminars in Thrombosis and Hemostasis
Jeffrey Van Eps, Joseph Fernandez-Moure, Fernando Cabrera, Xin Wang, Azim Karim, Bruna Corradetti, Paige Chan, Brian Dunkin, Ennio Tasciotti, Bradley Weiner, Warren Ellsworth
BACKGROUND: Recurrence after ventral hernia repair (VHR) remains a multifactorial problem still plaguing surgeons today. Some of the many contributing factors include mechanical strain, poor tissue-mesh integration, and degradation of matrices. The high recurrence rate witnessed with the use of acellular dermal matrices (ADM) for definitive hernia repair has reduced their use largely to bridging repair and breast reconstruction. Modalities that improve classic cellular metrics of successful VHR could theoretically result in improved rates of hernia recurrence; autologous platelet-rich plasma (PRP) may represent one such tool, but has been underinvestigated for this purpose...
August 2016: Surgical Endoscopy
Janine van Loon, Abbas Dehghan, Tang Weihong, Stella Trompet, Wendy L McArdle, Folkert F W Asselbergs, Ming-Huei Chen, Lorna M Lopez, Jennifer E Huffman, Frank W G Leebeek, Saonli Basu, David J Stott, Ann Rumley, Ron T Gansevoort, Gail Davies, James J F Wilson, Jacqueline C M Witteman, Xiting Cao, Anton J M de Craen, Stephan J L Bakker, Bruce M Psaty, John M Starr, Albert Hofman, J Wouter Jukema, Ian J Deary, Caroline Hayward, Pim van der Harst, Gordon D O Lowe, Aaron R Folsom, David P Strachan, Nicolas Smith, Moniek P M de Maat, Christopher O'Donnell
Low von Willebrand factor (VWF) levels are associated with bleeding symptoms and are a diagnostic criterion for von Willebrand disease, the most common inherited bleeding disorder. To date, it is unclear which genetic loci are associated with reduced VWF levels. Therefore, we conducted a meta-analysis of genome-wide association studies to identify genetic loci associated with low VWF levels. For this meta-analysis, we included 31 149 participants of European ancestry from 11 community-based studies. From all participants, VWF antigen (VWF:Ag) measurements and genome-wide single-nucleotide polymorphism (SNP) scans were available...
July 2016: European Journal of Human Genetics: EJHG
Karen I Maijer, Christiaan van der Leij, Maria J H de Hair, Sander W Tas, Mario Maas, Daniëlle M Gerlag, Paul P Tak, Cristina Lavini
OBJECTIVE: Analysis of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) using pharmacokinetic modeling (PKM) provides quantitative measures that mirror microvessel integrity and can be used as an objective marker of the level of synovial inflammation. The aim of this study was to investigate the PKM parameters K(trans) , kep , and ve in a prospective cohort of disease-modifying antirheumatic drug (DMARD)-naive patients with early arthritis, and to validate the results by assessing their correlation with the number of synovial endothelial cells (ECs)...
March 2016: Arthritis & Rheumatology
Alessandra Casonato, Elena Pontara, Margherita Morpurgo, Francesca Sartorello, Philip G De Groot, Maria G Cattini, Viviana Daidone, Luigi De Marco
Most circulating von Willebrand factor (VWF) is normally inactive and incapable of binding platelets, but numerous disorders may modify the proportion of active VWF. We explored active VWF levels in patients with von Willebrand disease (VWD) whose VWF had a higher affinity for platelet glycoprotein (GP)Ib, but different susceptibilities to ADAMTS13 and multimer patterns (9 patients lacking large multimers, 10 with a normal pattern); 12 patients with VWF C2362F and R1819_C1948delinsS mutations, which make VWF resistant to ADAMTS13 were also studied...
December 2015: British Journal of Haematology
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