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https://www.readbyqxmd.com/read/29879570/risk-factors-for-inhibitor-development-in-severe-hemophilia-a
#1
REVIEW
Isabella Garagiola, Roberta Palla, Flora Peyvandi
Although significant advances in hemophilia treatment have improved patient outcomes and quality of life, one of the greatest complications in severe hemophilia A is the development of anti-Factor VIII (FVIII) antibodies that inhibit FVIII activity in almost 30% of previously untreated patients (PUPs). Inhibitors make very difficult the management of patients and increase their morbidity and mortality reducing drastically their quality of life. Numerous studies have investigated the mechanisms leading to the development of FVIII inhibitors...
May 25, 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29674502/dissecting-the-pathophysiology-of-immune-thrombotic-thrombocytopenic-purpura-interplay-between-genes-and-environmental-triggers
#2
Johana Hrdinová, Silvia D'Angelo, Nuno A G Graça, Bogac Ercig, Karen Vanhoorelbeke, Agnès Veyradier, Jan Voorberg, Paul Coppo
Although outstanding progress has been made in comprehending the pathophysiology of thrombotic thrombocytopenic purpura, our understanding of the immunopathogenesis of the disease is only in its earlier stage. Anti-ADAMTS13 auto-antibodies were shown to block proteolysis of von Willebrand factor and/or induce ADAMTS13 clearance from the circulation. However, which immune cells are involved in the production of anti-ADAMTS13 autoantibodies and hence account for the remarkable efficacy of the B-cell depleting agents in this disease, remain to be identified...
April 19, 2018: Haematologica
https://www.readbyqxmd.com/read/29651379/molecular-cloning-and-characterization-of-pif-gene-from-pearl-mussel-hyriopsis-cumingii-and-the-gene-expression-analysis-during-pearl-formation
#3
Rui Zhang, Mengting Qin, Jie Shi, Lu Tan, Jiamin Xu, Zhenyan Tian, Yuhui Wu, Yuxuan Li, Yitian Li, Ning Wang
In the present study, the Pif gene of the freshwater pearl aquaculture mussel, Hyriopsis cumingii (HcPif) was successfully cloned and functionally characterized. The full sequence of HcPif gene consists of 3415 base pairs, which putatively encode two proteins, HcPif90 and HcPif80. A sequence analysis revealed that HcPif contained a von Willebrand factor type A domain and a chitin-binding domain, and shared many functional residues with other Pif homologues. A highly conserved sequence, FKGLDEIELML, at the C-terminus of Pif80s was identified as the key functional site...
April 2018: 3 Biotech
https://www.readbyqxmd.com/read/29179587/in-vitro-differentiation-of-progenitor-cells-isolated-from-juvenile-pig-hearts-expression-of-relevant-gene-and-protein-markers
#4
Simon Limbrecht Mogensen, Martin Krøyer Rasmussen, Niels Oksbjerg, Jette Feveile Young, Jens Rolighed Larsen
OBJECTIVES: Heart failure is a significant cause of mortality worldwide, and most current therapies treat only its symptoms. The results of cardiac stem cell research suggest a promising treatment option for heart failure, but there is currently an unmet demand for better research models. We have therefore, for the first time, isolated, expanded and differentiated progenitor cells obtained from juvenile pig hearts to use as a platform for cardiac stem cell research. DESIGN: Progenitor cells were isolated from the left ventricles of porcine hearts using collagenase enzymatic digestion and Percoll® -gradient centrifugation...
February 2018: Scandinavian Cardiovascular Journal: SCJ
https://www.readbyqxmd.com/read/29119855/two-novel-variants-of-uncertain-significance-in-gp9-associated-with-bernard-soulier-syndrome-are-they-true-mutations
#5
P Boisseau, C Debord, M Eveillard, A Quéméner, M Sigaud, M Giraud, P Talarmain, C Thomas, G Landeau, S Bezieau, B Pan Petesch, M C Béné, M Fouassier
Bernard-Soulier syndrome (BSS) is an autosomal recessive major thrombocytopathy, the symptoms of which are mainly marked by mucocutaneous bleeding. This rare disease, initially described in the 1970s, is the result of an abnormal formation of the glycoprotein complex Ib-IX-V (GP Ib-IX-V), a platelet receptor of von Willebrand factor. A large number of mutations, sometimes involving the GP9 gene, have been described as possibly responsible for the disease. We report here the case of a BSS patient who presented with persistent thrombocytopenia (31x109 /L) and decreased surface expression of GPIb-IX-V on large platelets with anisocytosis...
May 2018: Platelets
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
#6
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/28872471/lung-xenotransplantation
#7
REVIEW
Hisashi Sahara, Hironosuke Watanabe, Thomas Pomposelli, Kazuhiko Yamada
PURPOSE OF REVIEW: This review describes the most recent progress in xeno lung transplantation (XLTx) to date. It describes the potential mechanisms of early xeno lung graft loss, as well as the latest therapeutic strategies to overcome them. RECENT FINDINGS: Using ex-vivo perfusion models of porcine lungs with human blood, the use of genetically modified pig lungs along with novel pharmaceutical approaches has recently been studied. Strategies that have demonstrated improved lung survival include the knockout of known xenoantigens (GalTKO and N-glycolylneuraminic acid-KO), genes that regulate complement activation (hCD46 and hCD55), as well as the inflammation/coagulation cascade (human leukocyte antigen-E, human thrombomodulin, human endothelial protein C receptor, hCD47, hCD39, hCD73 and heme oxygenase-1)...
December 2017: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/28686347/epo-modified-mscs-can-inhibit-asthmatic-airway-remodeling-in-an-animal-model
#8
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...
January 2018: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28000367/cell-adhesive-peptides-functionalized-on-cocr-alloy-stimulate-endothelialization-and-prevent-thrombogenesis-and-restenosis
#9
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...
April 2017: Journal of Biomedical Materials Research. Part A
https://www.readbyqxmd.com/read/27941341/sulodexide-reduces-the-proinflammatory-effect-of-serum-from-patients-with-peripheral-artery-disease-in-human-arterial-endothelial-cells
#10
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
https://www.readbyqxmd.com/read/27428891/how-much-do-we-really-know-about-von-willebrand-disease
#11
REVIEW
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
https://www.readbyqxmd.com/read/26706543/preparation-of-the-fast-setting-and-degrading-ca-si-mg-cement-with-both-odontogenesis-and-angiogenesis-differentiation-of-human-periodontal-ligament-cells
#12
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
https://www.readbyqxmd.com/read/26578432/decreased-hernia-recurrence-using-autologous-platelet-rich-plasma-prp-with-strattice%C3%A2-mesh-in-a-rodent-ventral-hernia-model
#13
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
https://www.readbyqxmd.com/read/26486471/genome-wide-association-studies-identify-genetic-loci-for-low-von-willebrand-factor-levels
#14
Janine van Loon, Abbas Dehghan, Tang Weihong, Stella Trompet, Wendy L McArdle, Folkert 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
https://www.readbyqxmd.com/read/26184520/analysis-of-circulating-dna-and-protein-biomarkers-to-predict-the-clinical-activity-of-regorafenib-and-assess-prognosis-in-patients-with-metastatic-colorectal-cancer-a-retrospective-exploratory-analysis-of-the-correct-trial
#15
Josep Tabernero, Heinz-Josef Lenz, Salvatore Siena, Alberto Sobrero, Alfredo Falcone, Marc Ychou, Yves Humblet, Olivier Bouché, Laurent Mineur, Carlo Barone, Antoine Adenis, Takayuki Yoshino, Richard M Goldberg, Daniel J Sargent, Andrea Wagner, Dirk Laurent, Michael Teufel, Michael Jeffers, Axel Grothey, Eric Van Cutsem
BACKGROUND: Tumour mutational status is an important determinant of the response of metastatic colorectal cancer to targeted treatments. However, the genotype of the tissue obtained at the time of diagnosis might not accurately represent tumour genotype after multiple lines of treatment. This retrospective exploratory analysis investigated the clinical activity of regorafenib in biomarker subgroups of the CORRECT study population defined by tumour mutational status or plasma protein levels...
August 2015: Lancet Oncology
https://www.readbyqxmd.com/read/26090873/laminin-peptide-immobilized-hydrogels-modulate-valve-endothelial-cell-hemostatic-regulation
#16
Liezl Rae Balaoing, Allison Davis Post, Adam Yuh Lin, Hubert Tseng, Joel L Moake, K Jane Grande-Allen
Valve endothelial cells (VEC) have unique phenotypic responses relative to other types of vascular endothelial cells and have highly sensitive hemostatic functions affected by changes in valve tissues. Furthermore, effects of environmental factors on VEC hemostatic function has not been characterized. This work used a poly(ethylene glycol) diacrylate (PEGDA) hydrogel platform to evaluate the effects of substrate stiffness and cell adhesive ligands on VEC phenotype and expression of hemostatic genes. Hydrogels of molecular weights (MWs) 3...
2015: PloS One
https://www.readbyqxmd.com/read/25429217/incorporation-of-osteogenic-and-angiogenic-small-interfering-rnas-into-chitosan-sponge-for-bone-tissue-engineering
#17
Sen Jia, Xinjie Yang, Wen Song, Lei Wang, Kaixiu Fang, Zhiqiang Hu, Zihui Yang, Chun Shan, Delin Lei, Bin Lu
Engineered bone substitutes are being extensively explored in response to growing demand. However, the angiogenesis that occurs during bone formation is often overlooked in scaffold design. In this novel study, we incorporated two small interfering RNAs (siRNAs), ie, small interfering RNA targets casein kinase 2 interaction protein 1 (siCkip-1) and small interfering RNA targets soluble VEGF receptor 1 (siFlt-1), which can promote osteogenesis and angiogenesis, into a chitosan sponge. This scaffold could maintain siRNAs for over 2 weeks in neutral phosphate-buffered saline and degraded rapidly in the presence of lysozyme...
2014: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/25405250/role-of-p-selectin-and-p-selectin-glycoprotein-ligand-1-interaction-in-the-induction-of-tissue-factor-expression-on-human-platelets-after-incubation-with-porcine-aortic-endothelial-cells
#18
Mohamed B Ezzelarab, Yueh Wei Liu, Chih Che Lin, Cassandra Long, David Ayares, Anthony Dorling, David K C Cooper
Development of coagulation disorders remains a major challenge in pig-to-primate organ xenotransplantation. Our previous studies demonstrated that porcine aortic endothelial cells (pAEC) activate human platelets to express tissue factor (TF). In this study, we investigated the molecular interaction between human platelets and pAEC to identify possible targets for further genetic modification and/or systemic therapy. Human platelets were incubated with pAEC from wild-type (WT), α1,3-galactosyltransferase gene-knockout (GTKO), and GTKO pigs expressing human CD46, after which the platelets were analyzed for TF expression, TF mRNA level and TF function...
January 2014: Xenotransplantation
https://www.readbyqxmd.com/read/24975701/iti-choice-for-the-optimal-management-of-inhibitor-patients-from-a-clinical-and-pharmacoeconomic-perspective
#19
REVIEW
J Oldenburg, S K Austin, C M Kessler
The development of alloantibody inhibitors against factor VIII (FVIII) represents the most significant complication of haemophilia care. Inhibitors tend to develop early in the course of treatment in about 20-30% of patients with severe haemophilia who receive on-demand or prophylactic FVIII therapy. Many factors are associated with inhibitor formation, including disease severity, major FVIII gene defects, family history and non-Caucasian race, as well as age at first treatment, intensity of early treatment, use of prophylaxis and product choice...
September 2014: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/24705272/reversal-of-ischemic-cardiomyopathy-with-sca-1-stem-cells-modified-with-multiple-growth-factors
#20
Ning Li, Zeeshan Pasha, Muhammad Ashraf
BACKGROUND: We hypothesized that bone marrow derived Sca-1+ stem cells (BM Sca-1+) transduced with multiple therapeutic cytokines with diverse effects will induce faster angiomyogenic differentiation in the infarcted myocardium. METHODS AND RESULTS: BM Sca-1+ were purified from transgenic male mice expressing GFP. Plasmids encoding for select quartet of growth factors, i.e., human IGF-1, VEGF, SDF-1α and HGF were prepared and used for genetic modification of Sca-1+ cells (GFSca-1+)...
2014: PloS One
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