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Vamsee D Myneni, Aisha Mousa, Mari T Kaartinen
F13A1 gene, which encodes for Factor XIII-A blood clotting factor and a transglutaminase enzyme, was recently identified as a potential causative gene for obesity in humans. In our previous in vitro work, we showed that FXIII-A regulates preadipocyte differentiation and modulates insulin signaling via promoting plasma fibronectin assembly into the extracellular matrix. To understand the role of FXIII-A in whole body energy metabolism, here we have characterized the metabolic phenotype of F13a1-/- mice. F13a1-/- and F13a1+/+ type mice were fed chow or obesogenic, high fat diet for 20 weeks...
October 19, 2016: Scientific Reports
Kahori Nasu, Norio Hanafusa, Masaomi Nangaku
Bullous pemphigoid (BP) is an autoimmune blistering skin disorder characterized by circulating serum IgG antibodies against two hemidesmosomal proteins: BP180 and BP230. Fundamentally, immunosuppressive therapies are administered to treat this disease, but plasmapheresis can be added for refractory patients. We experienced the case of a 63-year-old patient with refractory BP for which we administered double filtration plasmapheresis (DFPP). His skin lesions improved along with decreased IgG BP180 antibodies, but factor XIII (FXIII) and fibrinogen were also reduced by DFPP repetition...
October 6, 2016: Journal of Clinical Apheresis
M M Tomasiak-Lozowska, T Misztal, T Rusak, J Branska-Januszewska, A Bodzenta-Lukaszyk, M Tomasiak
The aim of this study was to assess whether steroid-naïve asthma modulates hemostasis. We evaluated the clot retraction rate (CRR), fibrinolysis rate (FR), clot density (CD) (by confocal microscopy), plasma levels of plasminogen activator inhibitor (PAI-1), and factor XIII (FXIII), NO in exhaled breath (FENO ), spirometry (FEV1 ) and eosinophil count (EOS) in 36 patients with allergic, steroid-naïve asthma and in 34 healthy controls. We observed significantly (P < 0.001) reduced CRR, FR, and FEV1 and increased FENO , EOS, PAI-1, FXIII, and CD in patients with asthma compared with controls...
September 23, 2016: Allergy
M Martinuzzo, L Barrera, D Altuna, F Tisi Baña, J Bieti, Q Amigo, M D'Adamo, M S López, J Oyhamburu, J C Otaso
BACKGROUND: Homozygous or double heterozygous factor XIII (FXIII) deficiency is characterized by soft tissue hematomas, intracranial and delayed spontaneous bleeding. Alterations of thromboelastography (TEG) parameters in these patients have been reported. The aim of the study was to show results of TEG, TEG Lysis (Lys 60) induced by subthreshold concentrations of streptokinase (SK), and to compare them to the clot solubility studies results in samples of a 1-year-old girl with homozygous or double heterozygous FXIII deficiency...
2016: Mediterranean Journal of Hematology and Infectious Diseases
Majid Naderi, Mehran Karimi, Maryam Sadat Hosseini, Es Hagh Moradi, Morteza Shamsizadeh, Akbar Dorgalaleh
Factor XIII deficiency (FXIIID) is an extremely rare hemorrhagic disorder with a prevalence of 1/3-5 million. Management of disease is performed by fresh frozen plasma (FFP), Cryoprecipitate (CP) or FXIII concentrate (Fibrogammin P®). Our objective was to assess safety and effectiveness of Fibrogammin P® in patients with FXIIID. For this purpose we designed this long-term follow up study on a large group of patients with FXIIID. This prospective study was conducted on 213 patients with FXIIID since 2009 to 2013...
2016: Iranian Journal of Pharmaceutical Research: IJPR
Ching-Lin Hsieh, Eric Chang, Andrew Tseng, Christopher Ptak, Li-Chen Wu, Chun-Li Su, Sean P McDonough, Yi-Pin Lin, Yung-Fu Chang
The coagulation system provides a primitive but effective defense against hemorrhage. Soluble fibrinogen (Fg) monomers, composed of α, β and γ chains, are recruited to provide structural support for the formation of a hemostatic plug. Fg binds to platelets and is processed into a cross-linked fibrin polymer by the enzymatic clotting factors, thrombin and Factor XIII (FXIII). The newly formed fibrin-platelet clot can act as barrier to protect against pathogens from entering the bloodstream. Further, injuries caused by bacterial infections can be confined to the initial wound site...
September 2016: PLoS Neglected Tropical Diseases
Hideki Tatsukawa, Hong Hong Liu, Shota Oba, Noriho Kamiya, Yoichi Nakanishi, Kiyotaka Hitomi
Transglutaminases (TGs) comprise a protein family in which the members catalyze the formation of isopeptide bonds between glutamine and lysine residues in various proteins. Expression studies on its three major members, FXIII, TG1, and TG2, have been performed in a relatively large number of mammalian tissues in comparison with those on the other isozymes. We previously identified a highly reactive substrate peptide, including glutamine, for each isozyme from a phage display library and developed a method for detecting isozyme-specific activities by incorporating a labeled substrate peptide into lysine residues of proteins...
September 2, 2016: Amino Acids
Peter Taylor, Emelyn Salazar, Mariana Barrios, Ana María Salazar, María-Jesús Abad, Izaskun Urdanibia, David Shealy, Carmen Luisa Arocha-Piñango, Belsy Guerrero
INTRODUCTION: Contact with the caterpillar of Lonomia achelous causes a hemorrhagic syndrome in humans prompted by two processes, an initial mild DIC that is later masked by overwhelming fibrinolytic activity. Although the venom affects both the hemostatic and inflammatory systems separately, it is not clear whether the hematological and hemostatic disturbances may in part be due to an indirect effect via inflammatory mediators. Here we report results on the crosstalk between these systems, particularly the effect of the pro-inflammatory cytokine TNF-α on hemostatic parameters...
October 2016: Toxicon: Official Journal of the International Society on Toxinology
James R Byrnes, Clare Wilson, Anthony M Boutelle, Chase B Brandner, Matthew J Flick, Helen Philippou, Alisa S Wolberg
Coagulation transglutaminase factor XIII (FXIII) exists in circulation as heterotetrameric proenzyme FXIII-A2B2 Effectively all FXIII-A2B2 circulates bound to fibrinogen, and excess FXIII-B2 circulates in plasma. The motifs that mediate interaction of FXIII-A2B2 with fibrinogen have been elusive. We recently detected reduced binding of FXIII-A2B2 to murine fibrinogen that has γ-chain residues 390-396 mutated to alanines (Fibγ(390-396A)). Here, we evaluated binding features using human components, including recombinant fibrinogen variants, FXIII-A2B2, and isolated FXIII-A2 and -B2 homodimers...
October 13, 2016: Blood
Manuel Carcao, Katsuyuki Fukutake, Aida Inbal, Bryce Kerlin, Riitta Lassila, Johannes Oldenburg, May-Lill Garly, Diane Nugent
Congenital factor XIII (FXIII) deficiency is a rare, autosomal recessive bleeding disorder with potentially life-threatening consequences. FXIII is composed of two subunits (A and B), and a deficiency or dysfunction of either can result in FXIII deficiency. Traditionally, FXIII deficiency has been managed by infusing plasma-derived products containing FXIII (fresh frozen plasma, cryoprecipitate, and plasma-derived FXIII concentrates), all of which contain both subunits. Despite the increased safety of plasma-derived products, concern remains regarding potential viral safety issues...
August 24, 2016: Seminars in Thrombosis and Hemostasis
Vahideh Rabani, Damien Montange, Siamak Davani
Lipid-rafts are defined as membrane microdomains enriched in cholesterol and glycosphingolipids within platelet plasma membrane. Lipid raft-mediated clot retraction requires factor XIII and other interacting proteins. The aim of this study was to investigate the proteins that interact with factor XIII in raft and non-raft domains of activated and non-activated platelet plasma membrane. By lipidomics analysis, we identified cholesterol- and sphingomyelin-enriched areas as lipid rafts. Platelets were activated by thrombin...
September 2016: Platelets
Caroline Ulfsdotter Nilsson, Karin Strandberg, Martin Engström, Peter Reinstrup
BACKGROUND: Several studies have described hypercoagulability in neurosurgery with craniotomy for brain tumor resection. In this study, hydroxyethyl starch (HES) 130/0.42 was used for hemodynamic stabilization and initial blood loss replacement. HES can induce coagulopathy with thromboelastographic signs of decreased clot strength. The aim of this study was to prospectively describe perioperative changes in coagulation during elective craniotomy for brain tumor resection with the present fluid regimen...
2016: Perioperative Medicine
X Ye, B Ye
Although the Val34Leu polymorphism in blood coagulation factor XIII-A (FXIII-A) has been implicated in the pathogenesis of intracerebral hemorrhage (ICH), the results of research conducted thus far have been inconclusive. In this meta-analysis, we have assessed the association between the FXIII-A Val34Leu polymorphism and ICH risk. Published reports pertaining to this association were retrieved from the PubMed database, and the data from these studies were pooled and statistically analyzed with Stata 12.0. Summary odds ratios (OR) and 95% confidence intervals (95%CI) were calculated according to a fixed-effect or a random-effect model (as appropriate)...
2016: Genetics and Molecular Research: GMR
Akbar Dorgalaleh, Shadi Tabibian, Vahideh Assadollahi, Morteza Shamsizadeh, Iraj Zareban, Shahrzad Soori, Maryam Daneshi
BACKGROUND: Deficiency of factor XIII (FXIII) is a rare bleeding disorder (RBD) affecting approximately 1 person per 2 million worldwide. Its life-threatening diatheses-umbilical cord bleeding, intracranial hemorrhage and central nervous system bleeding-present a significant challenge in both diagnosis and treatment. Confirming FXIII deficiency (FXIIID) is difficult and has been most commonly performed with the clot solubility test. Iran has limited resources and a rate of FXIIID 12-fold higher than the rest of the world, suggesting that in this country optimization of the clot solubility test is crucial...
November 2016: Laboratory Medicine
N Schäfer, A Driessen, U Bauerfeind, M Fröhlich, J Ofir, E K Stürmer, M Maegele
OBJECTIVES: To analyse which fibrinogen source may improve coagulation using an in vitro 33% dilutional coagulopathy model. BACKGROUND: Uncritical volume resuscitation in the context of trauma haemorrhage contributes to the iatrogenic arm of the acute trauma-induced coagulopathy through dilution and depletion of coagulation factors, with fibrinogen reaching critical levels first. MATERIALS AND METHODS: By using an experimental model of 33% dilutional coagulopathy, we have analysed which fibrinogen source may exert superior effects on improving haemocoagulative capacities and correcting depleted fibrinogen levels...
August 9, 2016: Transfusion Medicine
Piotr Kuta, Barbara Hauck-Dlimi, Julian Strobel, Robert Zimmermann, Reinhold Eckstein
BACKGROUND: Uncontrolled hemorrhage in polytrauma patients usually results in rapid need of blood products. Despite the shorter thawing times of microwave devices for heating fresh frozen plasma (FFP), their use has remained controversial, and just a few laboratory analyses have been published on this topic. The aim of this study was to analyse the quality of clotting factors immediately after thawing FFP with a microwave device and after 48-hour post thaw storage at 4 degrees C. METHODS: 24 FFP units of all four ABO blood groups (six of each blood group) were thawed with a Transfusio-therm 2000 and later stored at 4 degrees C for 48 hours...
2016: Clinical Laboratory
Sneha Gupta, Arijit Biswas, Mohammad Suhail Akhter, Christoph Krettler, Christoph Reinhart, Johannes Dodt, Andreas Reuter, Helen Philippou, Vytautas Ivaskevicius, Johannes Oldenburg
The activation and regulation of coagulation Factor XIII (FXIII) protein has been the subject of active research for the past three decades. Although discrete evidence exists on various aspects of FXIII activation and regulation a combinatorial structure/functional view in this regard is lacking. In this study, we present results of a structure/function study of the functional chain of events for FXIII. Our study shows how subtle chronological submolecular changes within calcium binding sites can bring about the detailed transformation of the zymogenic FXIII to its activated form especially in the context of FXIIIA and FXIIIB subunit interactions...
2016: Scientific Reports
M Gissel, K E Brummel-Ziedins, S Butenas, A E Pusateri, K G Mann, T Orfeo
BACKGROUND: Disruption of hydrogen ion homeostasis is a consequence of traumatic injury often associated with clinical coagulopathy. Mechanisms by which acidification of the blood lead to aberrant coagulation require further elucidation. OBJECTIVE: To examine the effects of acidified conditions on coagulation dynamics using in vitro models of increasing complexity. METHODS: Coagulation dynamics were assessed at pH 7.4 and 7.0 as follows: 1) Tissue factor (TF)-initiated coagulation proteome mixtures (± factor (F)XI, ± fibrinogen/FXIII), with reaction progress monitored as thrombin generation or fibrin formation; 2) Enzyme/inhibitor reactions; 3) TF-dependent or -independent clot dynamics in contact pathway inhibited blood via viscoelastometry...
July 19, 2016: Journal of Thrombosis and Haemostasis: JTH
P de Moerloose, J-F Schved, D Nugent
Rare coagulation disorders (RCDs) include the inherited deficiencies of fibrinogen, factor (F) II, FV, combined FV and VIII, FVII, FX, combined FVII and X, FXI, FXIII and combined congenital deficiency of vitamin K-dependent factors (VKCFDs). Despite their rarity, a deep comprehension of all these disorders is essential to really understand haemostasis. Indeed, even if they share some common features each RCD has some particularity which makes it unique. In this review, we focus on three disorders: fibrinogen, FVII and FXIII...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
Anne Thomas, Arijit Biswas, Johannes Dodt, Helen Philippou, Emma Hethershaw, Hans Juergen Ensikat, Vytautas Ivaskevicius, Johannes Oldenburg
Inherited defects of coagulation Factor XIII (FXIII) can be categorized into severe and mild forms based on their genotype and phenotype. Heterozygous mutations occurring in F13A1 and F13B genes causing mild FXIII deficiency have been reported only in the last few years primarily because the mild FXIII deficiency patients are often asymptomatic unless exposed to some kind of a physical trauma. However, unlike mutations causing severe FXIII deficiency, many of these mutations have not been comprehensively characterized based on expression studies...
October 2016: Human Mutation
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