4,736 results match your criteria Factor XIII


Factor XIII: what does it look like?

J Thromb Haemost 2019 Mar 18. Epub 2019 Mar 18.

Division of Clinical Laboratory Science, Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.

Coagulation factor XIII (FXIII) is a stepchild among clotting factors. As opposed to all other zymogenic clotting factors, it is not the precursor of a proteolytic enzyme but of a transglutaminase. It is of tetrameric structure consisting of two types of subunits (FXIII-A B ). Read More

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http://dx.doi.org/10.1111/jth.14431DOI Listing

Prediction of Ischemic Events after Percutaneous Coronary Intervention: Thrombelastography Profiles and Factor XIIIa Activity.

TH Open 2018 Apr;2(2):e173-e181

Krannert Institute of Cardiology, Indiana University School of Medicine, Indianapolis, Indiana, United States.

Background: High plasma fibrin clot strength (MA) measured by thrombelastography (TEG) is associated with increased risk of cardiac events after percutaneous coronary interventions (PCIs). Factor XIIIa (FXIIIa) cross-links soluble fibrin, shortens clot formation time (TEG-K), and increases final clot strength (MA).

Methods: We analyzed platelet-poor plasma from patients with previous PCI. Read More

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http://dx.doi.org/10.1055/s-0038-1645876DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6419750PMC

Molecular Imaging of Factor XIII Activity for the Early Detection of Mouse Coronary Microvascular Disease.

Theranostics 2019 20;9(5):1474-1489. Epub 2019 Feb 20.

Section of Cardiovascular Medicine, Yale University School of Medicine, New Haven, CT, USA.

Coronary microvascular disease (MVD) remains a major clinical problem due to limited mechanistic understanding and a challenging diagnosis. In the present study we evaluated the utility of targeted imaging of active factor XIII (FXIII) for detection of coronary MVD associated with thrombus. We hypothesized that a high specificity and sensitivity FXIII targeted radiolabeled probe can serve as a biomarker for cross-linked thrombi in the microvasculature, and thus an indicator for underlying coronary MVD. Read More

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http://dx.doi.org/10.7150/thno.29255DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6401499PMC
February 2019
2 Reads

Acquired haemophilia A and concomitant factor XIII consumption.

Haemophilia 2019 Mar 7. Epub 2019 Mar 7.

Department of Laboratory Medicine and Pathobiology, St. Michael's Hospital, Toronto, Ontario, Canada.

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http://dx.doi.org/10.1111/hae.13690DOI Listing

Factor XIII topology: organization of B subunits and changes with activation studied with single-molecule atomic force microscopy.

J Thromb Haemost 2019 Feb 17. Epub 2019 Feb 17.

Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.

Essentials Factor XIII is a heterotetramer with 2 catalytic A subunits and 2 non-catalytic B subunits. Structure of active and inactive factor XIII was studied with atomic force microscopy. Inactive factor XIII is made of an A globule and 2 flexible B subunits extending from it. Read More

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http://dx.doi.org/10.1111/jth.14412DOI Listing
February 2019
4 Reads

Coagulation factor XIII-A and activated XIII-A decrease in some deep vein thrombosis patients following catheter-directed thrombolysis.

Blood Coagul Fibrinolysis 2019 Feb 11. Epub 2019 Feb 11.

Michael Smith Laboratories, Department of Biochemistry and Molecular Biology, University of British Columbia.

: To assess the effect of tissue plasminogen activator administered during catheter-directed thrombolysis (CDT) on coagulation factor XIII (FXIII). Thrombolytic therapy carries significant risks, such as life-threatening bleeds. The mechanisms responsible for major bleeds and intracerebral hemorrhages during thrombolysis are not fully understood. Read More

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http://dx.doi.org/10.1097/MBC.0000000000000797DOI Listing
February 2019
3 Reads

Optimised methods (SDS/PAGE and LC-MS) reveal deamidation in all examined transglutaminase-mediated reactions.

FEBS Open Bio 2019 Feb 18;9(2):396-404. Epub 2019 Jan 18.

Research Department Covalab S.A.S Lyon France.

Transglutaminases (TGs) are a family of structurally and functionally related enzymes that catalyse calcium-dependent post-translational modifications of proteins through protein-protein crosslinking, amine incorporation, or deamidation. For many years deamidation mediated by TGs was considered to be a side reaction, but recently substrate-specific deamidations have been reported. Here we describe an optimised SDS/PAGE assay for the easy and rapid monitoring of the TG reaction with small peptides. Read More

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http://dx.doi.org/10.1002/2211-5463.12575DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6356169PMC
February 2019
2 Reads

Greenhouse gas emissions and soil organic matter dynamics in woody crop orchards with different irrigation regimes.

Sci Total Environ 2018 Dec 23;644:1429-1438. Epub 2018 Jul 23.

Dpto. Producción Vegetal, ETSIA, Universidad Politécnica de Cartagena, Paseo Alfonso XIII, 52, 30230 Cartagena, Spain.

Water scarcity in arid, semiarid and dry regions is a limiting factor for the development of sustainable agriculture. As a consequence, the adoption of new strategies such as regulated deficit irrigation (RDI) to reduce water and energy consumption will be essential. Decreases in irrigation water content may also have positive effects on soil C cycle. Read More

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http://dx.doi.org/10.1016/j.scitotenv.2018.06.398DOI Listing
December 2018
3 Reads

Recurrent venous thromboembolism patients form clots with lower elastic modulus than those formed by patients with non-recurrent disease.

J Thromb Haemost 2019 Feb 6. Epub 2019 Feb 6.

Leeds Thrombosis Collective, Department of Discovery and Translational Science, Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, UK.

Essentials Venous thromboembolism (VTE) recurrence leads to decreased clot elastic modulus in plasma. Recurrent VTE is not linked to changes in clot structure, fiber radius, or factor XIII activity. Other plasma components may play a role in VTE recurrence. Read More

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http://dx.doi.org/10.1111/jth.14402DOI Listing
February 2019
4 Reads

Efficacy of a new pathogen-reduced cryoprecipitate stored 5 days after thawing to correct dilutional coagulopathy in vitro.

Transfusion 2019 Feb 4. Epub 2019 Feb 4.

Department of Anaesthesia, Zurich University Children's Hospital, Zurich, Switzerland.

Background: Fibrinogen supplementation during bleeding restores clot strength and hemostasis. Cryoprecipitate, a concentrated source of fibrinogen, has prolonged preparation time for thawing, a short shelf life resulting in frequent wastage, and infectious disease risk. This in vitro study investigated the efficacy of a new pathogen-reduced cryoprecipitate thawed and stored at room temperature for 5 days (PR Cryo) to treat dilutional hypofibrinogenemia, compared to immediately thawed standard cryoprecipitate (Cryo) or fibrinogen concentrate (FC). Read More

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http://dx.doi.org/10.1111/trf.15157DOI Listing
February 2019
2 Reads
3.225 Impact Factor

On the early events of the calcium-induced activation of coagulation factor XIII-A and tissue transglutaminase: an in silico study.

J Biomol Struct Dyn 2019 Feb 1:1-16. Epub 2019 Feb 1.

b Division of Clinical Laboratory Science, Department of Laboratory Medicine, Faculty of Medicine , University of Debrecen , Debrecen , Hungary.

Both coagulation factor XIII-A (FXIII-A) and tissue transglutaminase (TG2) play distinctive and important roles in homeostasis by crosslinking proteins or peptides via isopeptide bonds. In this present study, a series of microsecond-long all-atom molecular dynamics (MD) simulations were carried out in order to reveal the dynamic, atomic-level events which may contribute to the activation of these proteins via the binding of calcium ions. In addition to previously conducted in vitro and crystallographic studies, further suggestions have been made concerning the calcium binding features of these enzymes. Read More

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http://dx.doi.org/10.1080/07391102.2019.1574604DOI Listing
February 2019
1 Read

Coagulopathy in Type 2 Diabetes Mellitus: pathological mechanisms and the role of Factor XIII-A single nucleotide polymorphisms.

Curr Diabetes Rev 2019 Jan 29. Epub 2019 Jan 29.

Department of Physiology, Faculty of Health Sciences, University of Pretoria. South Africa.

The prevalence of type 2 diabetes mellitus (T2DM) has quadrupled within three decades since 1980 affecting 422 million adults in 2016. It remains one of the most common non-communicable chronic diseases and underlying risk factor for cardiovascular diseases worldwide. There are different underlying mechanisms that play a role in the development of pathologies associated with the disease such as hyperglycaemia, oxidative stress, obesity, inflammation and hypercoagulation. Read More

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http://dx.doi.org/10.2174/1573399815666190130113328DOI Listing
January 2019
2 Reads

Segmental uniparental disomy as a rare cause of congenital severe factor XIII deficiency in a girl with only one heterozygous carrier parent.

Pediatr Hematol Oncol 2019 Jan 31:1-5. Epub 2019 Jan 31.

e Department of Pediatrics , National Taiwan University Hospital , Taipei , Taiwan.

Uniparental disomy (UPD) refers to a situation when a person inherits both homologs of a region or complete part of a chromosome from only one parent. Here, we present an unusual case of UPD in congenital severe factor (F) XIII deficiency. A 6-year-old girl experienced cephalhematoma and umbilical bleeding after birth and easy bruising, and postextraction bleeding since early infancy. Read More

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http://dx.doi.org/10.1080/08880018.2018.1546783DOI Listing
January 2019
2 Reads

Faster fibrin clot degradation characterizes patients with central pulmonary embolism at a low risk of recurrent peripheral embolism.

Sci Rep 2019 Jan 11;9(1):72. Epub 2019 Jan 11.

Krakow Centre for Medical Research and Technology, John Paul II Hospital, Krakow, Poland.

It is unclear whether thrombus location in pulmonary arteries is associated with particular clot characteristics. We assessed 156 patients following either central or peripheral pulmonary embolism (PE). Plasma clot lysis time, the rate of D-dimer release from plasma clots (D-D) with the maximum D-dimer concentration achieved (D-D), as well as fibrin formation on turbidimetry, plasma clot permeation, thrombin generation, and fibrinolytic parameters were measured 3-6 months after PE. Read More

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http://dx.doi.org/10.1038/s41598-018-37114-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329786PMC
January 2019
4 Reads

A Unique Factor XIII Mutation in Southeastern Iran with an Unexpectedly High Prevalence: Khash Factor XIII.

Semin Thromb Hemost 2019 Feb 10;45(1):43-49. Epub 2019 Jan 10.

Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran.

Congenital factor XIII (FXIII) deficiency is an extremely rare hemorrhagic disorder characterized by a deficiency of FXIII and associated with a high rate of morbidity and mortality. The disorder is more frequent in Iran, especially in Khash, a city in the southeast of the country. As identified in the current report, the prevalence of FXIII deficiency in this city is 1 homozygote per approximately 500 population (which is ∼4,000 times higher than the worldwide prevalence) with 3. Read More

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http://dx.doi.org/10.1055/s-0038-1676580DOI Listing
February 2019
5 Reads
1 Citation
3.876 Impact Factor

Transglutaminase diseases: from biochemistry to the bedside.

FASEB J 2019 Jan;33(1):3-12

Molecular Cardiology and Biophysics Division, Victor Chang Cardiac Research Institute, Darlinghurst, New South Wales, Australia.

In humans, 9 members of the transglutaminase (TG) family have been identified, of which 8 [factor XIII (FXIII)A and TG1-TG7] catalyze post-translational protein-modifying reactions, and 1 does not (protein 4.2). The TG enzymatic activities considered in our discussion of human disease include deamidation of glutamine (Gln) residues, amine incorporation into Gln residues, and protein crosslinking. Read More

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http://dx.doi.org/10.1096/fj.201801544RDOI Listing
January 2019
2 Reads

Pro-coagulant haemostatic factors for the prevention and treatment of bleeding in people without haemophilia.

Cochrane Database Syst Rev 2018 12 24;12:CD010649. Epub 2018 Dec 24.

John Radcliffe Hospital, Oxford, UK, OX3 9DU.

Background: Some hospital patients may be at risk of or may present with major bleeding. Abnormalities of clotting (coagulation) are often recorded in these people, and the traditional management has been with transfusions of blood components, either to prevent bleeding (prophylactic) or to treat bleeding (therapeutic). There is growing interest in the use of targeted therapies with specific pro-coagulant haemostatic (causing bleeding to stop and to keep blood within a damaged blood vessel) factor concentrates in place of plasma. Read More

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http://dx.doi.org/10.1002/14651858.CD010649.pub2DOI Listing
December 2018
3 Reads

Live Confocal Visualization of FXIII Activity.

Clin Lab 2018 Oct;64(11)

In addition to its important functions in angiogenesis, wound healing, bone biology, immunology, pregnancy, and adipogenesis, transglutaminase factor XIII is an outstanding determinant of clot characteristics. Factor XIII mediates clot stability by covalent cross-linking of fibrin-fibrils and inhibition of fibrinolysis. For decades it was assumed that factor XIII mediates the formation of a 3-dimensional net structure of the clot. Read More

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http://dx.doi.org/10.7754/Clin.Lab.2018.180601DOI Listing
October 2018
3 Reads

Transglutaminases in Monocytes and Macrophages.

Med Sci (Basel) 2018 Dec 11;6(4). Epub 2018 Dec 11.

Division of Biomedical Sciences, Faculty of Dentistry, McGill University, Montreal, QC, H3A 0C7, Canada.

Macrophages are key players in various inflammatory disorders and pathological conditions via phagocytosis and orchestrating immune responses. They are highly heterogeneous in terms of their phenotypes and functions by adaptation to different organs and tissue environments. Upon damage or infection, monocytes are rapidly recruited to tissues and differentiate into macrophages. Read More

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http://www.mdpi.com/2076-3271/6/4/115
Publisher Site
http://dx.doi.org/10.3390/medsci6040115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6313455PMC
December 2018
13 Reads

Probable Roles of Coagulation Cascade and Fibrinolysis System in the Development of Allergic Rhinitis.

Am J Rhinol Allergy 2019 Mar 6;33(2):137-144. Epub 2018 Dec 6.

3 Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea.

Background: Dysregulation of the coagulation cascade and fibrinolysis system may play an etiologic role in many diseases. Allergic diseases such as bronchial asthma, atopic dermatitis, and conjunctivitis are also associated with fibrin accumulation caused by a change in hemostasis. However, only a few studies have dealt with the relationship between allergic rhinitis (AR) and the coagulation system. Read More

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http://dx.doi.org/10.1177/1945892418816015DOI Listing
March 2019
4 Reads

Effect of early administration of coagulation factor XIII on fistula after pancreatic surgery: the FIPS randomized controlled trial.

Langenbecks Arch Surg 2018 Dec 30;403(8):933-940. Epub 2018 Nov 30.

Department of Hepatobiliary-Pancreatic Surgery, Japanese Foundation for Cancer Research, Cancer Institute Hospital, 3-8-31 Ariake, Koto-ku, Tokyo, 135-8550, Japan.

Purpose: The administration of exogenous factor XIII (FXIII) is reportedly effective for fistula closure in patients with a low plasma FXIII level. This study was performed to analyze the effect of early administration of exogenous FXIII on postoperative pancreatic fistula (POPF).

Methods: A single-center randomized controlled, open-label, parallel group, superiority trial was conducted from October 2015 to August 2016 in Japan. Read More

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http://link.springer.com/10.1007/s00423-018-1736-4
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http://dx.doi.org/10.1007/s00423-018-1736-4DOI Listing
December 2018
11 Reads

FranceCoag: a 22-year prospective follow-up of the national French cohort of patients with inherited bleeding disorders.

Eur J Epidemiol 2018 Dec 5. Epub 2018 Dec 5.

Haemophilia Treatment Centre, APHM, Children Hospital La Timone, Aix Marseille University, INSERM, INRA, C2VN, Marseille, France.

FranceCoag is an ongoing open prospective multicentre cohort project aimed at improving epidemiological knowledge about inherited bleeding disorders in France. The main objective of this article was to evaluate the project's progress as of the 30th December 2016. Between 1994 and this date, of the 10,047 patients included in the study, 384 (3. Read More

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http://link.springer.com/10.1007/s10654-018-0468-7
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http://dx.doi.org/10.1007/s10654-018-0468-7DOI Listing
December 2018
8 Reads

Factor-XIII activity in patients with mild to moderate ulcerative colitis and active bleeding: a prospective observational study.

BMC Res Notes 2018 Dec 4;11(1):853. Epub 2018 Dec 4.

Practice for Digestive and Metabolic Diseases, Nordstr. 21, 04105, Leipzig, Germany.

Objective: Coagulation factor XIII plays a key role in fibrin clot stabilization and epithelial healing. Under chronic inflammatory conditions involving bleeding and an activation of the coagulation cascade, the FXIIIa inversely correlate with disease activity. We assumed that FXIIIa could be a predictor of severity in patients with ulcerative colitis (UC). Read More

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http://dx.doi.org/10.1186/s13104-018-3963-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6278029PMC
December 2018
5 Reads

The impact of antenatal factor XIII levels on postpartum haemorrhage: a prospective observational study.

Arch Gynecol Obstet 2019 Feb 3;299(2):421-430. Epub 2018 Dec 3.

Department of Obstetrics, Charité-University Medical Center, Augustenburger Platz 1, 13353, Berlin, Germany.

Purpose: Postpartum haemorrhage (PPH) is a leading cause of maternal mortality and morbidity. Our aim was to investigate the relationships between antenatal factor XIII (FXIII), fibrinogen levels, and blood loss at childbirth.

Methods: This prospective observational study evaluated an unselected cohort of pregnant women admitted for intended vaginal deliveries of singletons at term. Read More

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http://dx.doi.org/10.1007/s00404-018-4980-5DOI Listing
February 2019
2 Reads

Acquired Factor Xiii Deficiency: An Uncommon But Easily Missed Cause Of Severe Bleeding

Ir Med J 2018 May 10;111(5):757. Epub 2018 May 10.

National Coagulation Centre, St. James’s Hospital, Dublin, Ireland

Factor XIII (FXIII) is a plasma clotting protein involved in clot stabilization. Severe FXIII deficiency may present with severe, even fatal bleeding. Critically however, routine coagulation assays may be normal and only specific FXIII assays will detect the abnormality. Read More

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May 2018
1 Read

Let's cross-link: diverse functions of the promiscuous cellular transglutaminase factor XIII-A.

J Thromb Haemost 2019 Jan;17(1):19-30

Aberdeen Cardiovascular & Diabetes Centre, School of Medicine, Medical Sciences and Nutrition, Institute of Medical Sciences, University of Aberdeen, Aberdeen, UK.

Essentials Plasma Factor XIII, a heterodimer of A and B subunits FXIIIA B , is a transglutaminase enzyme with a well-established role in haemostasis. Cells of bone marrow and mesenchymal lineage express the FXIII-A gene (F13A1) that encodes the cellular form of the transglutaminase, a homodimer of the A subunits, FXIII-A. FXIII-A was presumed to function intracellularly, however, several lines of evidence now indicate that FXIII-A is externalised by an as yet unknown mechanism This review describes the mounting evidence that FXIII-A is a diverse transglutaminase with many intracellular and extracellular substrates that can participate in an array of biological processes SUMMARY: Factor XIII is a tranglutaminase enzyme that catalyzes the formation of ε-(γ-glutamyl)lysyl isopeptide bonds in protein substrates. Read More

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http://dx.doi.org/10.1111/jth.14348DOI Listing
January 2019
2 Reads

The unusual pattern of hereditary bleeding disorders in the province of Newfoundland and Labrador-Canada's most Eastern Province.

Transfus Apher Sci 2018 Dec 30;57(6):713-716. Epub 2018 Oct 30.

Memorial University of Newfoundland Primary Healthcare Research Unit, Health Sciences Centre, 300 Prince Philip Dr., St. John's, NL A1B 3 V6, Canada. Electronic address:

Newfoundland and Labrador (NL), the most eastern province of Canada, is characterized by a unique topography and pattern of settlement. The current population is descended from a small founding population of indigenous Innu, Inuit and Mi'kmaq and an estimated 28,000 settlers. These settlers originated from Southwest England and Southeast Ireland and came to invest and work in one of the world's richest fisheries. Read More

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http://dx.doi.org/10.1016/j.transci.2018.10.010DOI Listing
December 2018
14 Reads

Fusion of Factor IX to Factor XIII-B Sub-Unit Improves the Pharmacokinetic Profile of Factor IX.

Thromb Haemost 2018 Dec 19;118(12):2053-2063. Epub 2018 Nov 19.

EA4609-Hemostase et Cancer, Universite Claude Bernard Lyon I, Lyon, France.

Prophylaxis is currently considered the optimal care for severe haemophilia. For patients and their families one of the major difficulties with prophylaxis is the need for frequent venipunctures. The half-life of standard factor IX (FIX) concentrates is approximately 18 hours, which requires 2 or 3 intravenous infusions per week to achieve bleeding prevention in patients with severe haemophilia B. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1675787
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http://dx.doi.org/10.1055/s-0038-1675787DOI Listing
December 2018
12 Reads

Environmental impact assessment of industrial activities on heavy metals distribution in street dust and soil.

Chemosphere 2019 Feb 12;217:695-705. Epub 2018 Nov 12.

Sustainable Use, Management and Reclamation of Soil and Water Research Group, Universidad Politecnica de Cartagena, Paseo Alfonso XIII, 48, 30203, Cartagena, Murcia, Spain. Electronic address:

Street dust and soil are important materials for evaluating the contaminants level in industrial areas. Detailed size-resolved distribution of metal(loid)s in street dusts and soils influenced by industrial activities has rarely been investigated. This study was carried out to understand how industrialization might affect the size distribution of metal(loid)s concentration and contamination level in the street dust and soil from Murcia, southern Spain. Read More

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http://dx.doi.org/10.1016/j.chemosphere.2018.11.045DOI Listing
February 2019
13 Reads

A high titer of acquired factor V inhibitor in a hemodialysis patient who developed arterial thrombosis.

Int J Hematol 2019 Feb 16;109(2):214-220. Epub 2018 Nov 16.

Japanese Collaborative Research Group on Autoimmune Coagulation Factor Deficiencies (JCRG supported by the Japanese Ministry of Health, Labor and Welfare), Yamagata, Japan.

An 87-year-old man with diabetes mellitus was admitted to control recurrent bleeding from hemodialysis puncture sites. He was a smoker and had been diagnosed with arteriosclerosis obliterans. His PT and APTT were markedly prolonged, and all coagulation factors were markedly decreased (factor V [FV] activity < 1%) or below the measurement threshold, with the exception of fibrinogen and factor XIII. Read More

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http://link.springer.com/10.1007/s12185-018-2561-9
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http://dx.doi.org/10.1007/s12185-018-2561-9DOI Listing
February 2019
19 Reads

Inherited genetic predispositions in F13A1 and F13B genes predict abdominal adhesion formation: identification of gender prognostic indicators.

Sci Rep 2018 Nov 16;8(1):16916. Epub 2018 Nov 16.

Department of Morphology, Surgery & Experimental Medicine, University of Ferrara and Vascular Diseases Centre, Unit of Translational Surgery, University-Hospital of Ferrara, via Aldo Moro 8, 44124, Cona-Ferrara, Italy.

Abdominal adhesions (AA) account for the most common complication of peritoneal surgery with bowel obstruction being the severest problem in the absence of effective predicting biomarkers. Anti-AA-barriers or adhesiolysis did not completely prevent bowel obstruction, although there is evidence they might reduce related complications requiring reoperation. In addition, gender-related predispositions have not been adequately investigated. Read More

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http://dx.doi.org/10.1038/s41598-018-35185-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6240050PMC
November 2018
5 Reads

Acquired factor XIII deficiency: A review.

Transfus Apher Sci 2018 Dec 30;57(6):724-730. Epub 2018 Oct 30.

St. Michael's Hospital, Room 2-007G Core Lab, 30 Bond Street, Toronto, ON, M5B 1W8, Canada. Electronic address:

Acquired factor XIII (FXIII) deficiency is a rare bleeding disorder that can manifest with spontaneous or delayed life-threatening hemorrhage. Causes of acquired deficiency include immune-mediated inhibition, as well as non-immune FXIII hyperconsumption or hyposynthesis. The occurrence of acquired FXIII deficiency can be idiopathic or may be associated with comorbidities, such as malignancies or autoimmune disorders. Read More

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https://www.researchgate.net/profile/Behrouz_Mansouri/public
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https://linkinghub.elsevier.com/retrieve/pii/S14730502183043
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http://dx.doi.org/10.1016/j.transci.2018.10.013DOI Listing
December 2018
15 Reads

Single-donor spray-dried plasma.

Transfusion 2019 Feb 15;59(2):707-713. Epub 2018 Nov 15.

Velico Medical, Inc., Beverly, Massachusetts.

Background: Dried plasma is logistically superior for hemostasis management because it can be transported and stored under nonfrozen conditions and quickly reconstituted at the point of care, enabling prehospital administration. Velico Medical has developed a spray-drying system to be integrated into routine blood center work streams for spray drying single donor plasma units. This study compared the quality of the spray-dried plasma (on-demand plasma [ODP]) with fresh frozen plasma (FFP). Read More

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http://dx.doi.org/10.1111/trf.15035DOI Listing
February 2019
5 Reads

Proline 36 of the Factor XIII Activation Peptide Plays a Crucial Role in Substrate Recognition and Zymogen Activation.

Thromb Haemost 2018 Dec 12;118(12):2037-2045. Epub 2018 Nov 12.

Experimental Haemostasis Group, Department for BioMedical Research, University of Bern, Bern, Switzerland.

The activation peptide of blood coagulation factor XIII (AP-FXIII) has important functions in stabilizing the FXIII-A dimer and regulating FXIII activation. Contributions of many of its 37 amino acids to these functions have been described. However, the role of proline 36, which is adjacent to the thrombin cleavage site at Arg37, has not yet been studied in detail. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1675600
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http://dx.doi.org/10.1055/s-0038-1675600DOI Listing
December 2018
14 Reads

Chronic liver injury drives non-traditional intrahepatic fibrin(ogen) crosslinking via tissue transglutaminase.

J Thromb Haemost 2019 Jan 10;17(1):113-125. Epub 2018 Dec 10.

Institute for Integrative Toxicology, Michigan State University, East Lansing, MI, USA.

Essentials Fibrin clots are often implicated in the progression of liver fibrosis. Liver fibrosis was induced in transgenic mice with defects in clot formation or stabilization. Liver fibrosis and fibrin(ogen) deposition do not require fibrin polymerization or factor XIIIa. Read More

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http://doi.wiley.com/10.1111/jth.14330
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http://dx.doi.org/10.1111/jth.14330DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322974PMC
January 2019
13 Reads

Crosslinked clots formed independently of factor XIII and without fibrinogen-to-fibrin conversion - is this a liver-specific phenomenon?

Authors:
T Lisman

J Thromb Haemost 2019 Jan 10;17(1):110-112. Epub 2018 Dec 10.

Surgical Research Laboratory and Section of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.

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http://doi.wiley.com/10.1111/jth.14328
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http://dx.doi.org/10.1111/jth.14328DOI Listing
January 2019
16 Reads

Coagulation factor XIIIa cross-links amyloid β into dimers and oligomers and to blood proteins.

J Biol Chem 2019 Jan 8;294(2):390-396. Epub 2018 Nov 8.

From the Michael Smith Laboratories, and Centre for Blood Research, University of British Columbia, Vancouver, British Columbia, Canada V6T 1Z4,

In cerebral amyloid angiopathy (CAA) and Alzheimer's disease (AD), the amyloid β (Aβ) peptide deposits along the vascular lumen, leading to degeneration and dysfunction of surrounding tissues. Activated coagulation factor XIIIa (FXIIIa) covalently cross-links proteins in blood and vasculature, such as in blood clots and on the extracellular matrix. Although FXIIIa co-localizes with Aβ in CAA, the ability of FXIIIa to cross-link Aβ has not been demonstrated. Read More

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http://dx.doi.org/10.1074/jbc.RA118.005352DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333891PMC
January 2019
12 Reads

Novel Factor XIII variant identified through whole-genome sequencing in a child with intracranial hemorrhage.

Cold Spring Harb Mol Case Stud 2018 Dec 17;4(6). Epub 2018 Dec 17.

Rady Children's Institute of Genomic Medicine, University of California, San Diego, California 92123, USA.

Pediatric stroke can be either hemorrhagic or ischemic, with ∼5% of hemorrhagic strokes being caused by genetic coagulopathies. We report an 8 mo old presenting with a hemorrhagic stroke caused by severe Factor XIII deficiency (OMIM # 613225) in whom rapid whole-genome sequencing identified a novel variant in the gene c.1352_1353delAT (p. Read More

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http://dx.doi.org/10.1101/mcs.a003525DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6318776PMC
December 2018
3 Reads

Management of rare coagulation disorders in 2018.

Transfus Apher Sci 2018 Dec 30;57(6):705-712. Epub 2018 Oct 30.

Bleeding Disorders and Thrombosis Program, Cohen Children's Medical Center of New York and Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, United States.

Rare bleeding disorders (RBDs) comprise inherited deficiencies of factors I (fibrinogen), II (prothrombin), V, VII, X, XI, and XIII as well as combined factor V + VIII and vitamin K-dependent factors. They represent 3-5% of all congenital bleeding disorders and are usually transmitted as autosomal recessive traits. These disorders often manifest during childhood and have varied clinical presentations from mucocutaneous bleeding to life-threatening symptoms such as central nervous system and gastrointestinal bleeding. Read More

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http://dx.doi.org/10.1016/j.transci.2018.10.009DOI Listing
December 2018
6 Reads

Central Hypoxia Elicits Long-Term Expression of the Lung Motor Pattern in Pre-metamorphic Lithobates Catesbeianus.

Adv Exp Med Biol 2018 ;1071:75-82

Department of Pediatrics, Université Laval, Québec, QC, Canada.

During vertebrate development, the neural networks underlying air-breathing undergo changes in connectivity and functionality, allowing lung ventilation to emerge. Yet, the factors regulating development of these critical homeostatic networks remain unresolved. In amphibians, air-breathing occurs sporadically prior to metamorphosis. Read More

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http://dx.doi.org/10.1007/978-3-319-91137-3_9DOI Listing
January 2018
3 Reads

Anti Müllerian Hormone: More than a biomarker of female reproductive function.

J Gynecol Obstet Hum Reprod 2019 Jan 22;48(1):19-24. Epub 2018 Oct 22.

Department of Reproductive Medicine and Fertility Preservation, Hôpital Antoine Béclère, Hôpitaux Universitaires Paris Sud, Assistance Publique - Hôpitaux de Paris, Clamart 92140, France; Université Paris-Sud, Université Paris Saclay, Le Kremlin Bicêtre 94276, France; Inserm U1133 Université Paris Diderot, Paris 75013, France. Electronic address:

Anti-Müllerian hormone (AMH), known for its role during sexual differentiation, is a dimeric glycoprotein that belongs to the transforming growth factor-β (TGF-β) family. AMH has recently been identified as a reliable marker of ovarian reserve that can help predict early ovarian follicle loss and menopause onset. AMH levels also reflect the effects of damaging gynecologic surgeries or gonadotoxic treatments such as chemotherapy on ovarian reserve. Read More

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http://dx.doi.org/10.1016/j.jogoh.2018.10.015DOI Listing
January 2019
24 Reads

Rare congenital bleeding disorders.

Ann Transl Med 2018 Sep;6(17):331

Italian National Blood Centre, National Institute of Health, Rome, Italy.

The rare congenital bleeding disorders are a heterogeneous group of diseases which include deficiencies of fibrinogen, prothrombin and factors V, V + VIII, VII, X, XI and XIII. They are usually transmitted as autosomal recessive disorders, and the prevalence of the severe forms ranges from one case in 500,000 for factor VII up to one in 2,000,000 for factor XIII in the general population. Patients with rare congenital bleeding disorders may have a broad spectrum of clinical symptoms, ranging from mucocutaneous bleeding to life-threatening haemorrhages, such as those occurring in the central nervous system. Read More

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http://atm.amegroups.com/article/view/21055/20739
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http://dx.doi.org/10.21037/atm.2018.08.34DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174186PMC
September 2018
6 Reads

New Biomarkers for Prediction of Disseminated Intravascular Coagulation in Patients With Sepsis.

Clin Appl Thromb Hemost 2018 Oct 10:1076029618804078. Epub 2018 Oct 10.

5 Department of Disaster and Emergency Medicine. Kobe University Graduate School of Medicine, Hyogo, Japan.

Complication of disseminated intravascular coagulation (DIC) is a determinant of the prognosis for patients with sepsis. The purpose of this study was to find DIC-related peptides in blood for prediction and early diagnosis of DIC in patients with sepsis. The participants were 20 patients with sepsis (age: 68. Read More

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http://dx.doi.org/10.1177/1076029618804078DOI Listing
October 2018
8 Reads

Impaired factor XIII activation in patients with congenital afibrinogenemia.

Haematologica 2019 Mar 27;104(3):e111-e113. Epub 2018 Sep 27.

Division of Angiology and Haemostasis, Faculty of Medicine, University Hospitals of Geneva, Switzerland.

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http://dx.doi.org/10.3324/haematol.2018.203901DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395312PMC
March 2019
5 Reads

Use of Genie 2000 and Excel VBA to correct for γ-ray interference in the determination of NORM building material activity concentrations.

Appl Radiat Isot 2018 Dec 15;142:1-7. Epub 2018 Sep 15.

Eduardo Torroja Institute for Construction Sciences, IETcc-CSIC, Madrid, Spain.

The γ-radiation emitted by building materials is calculated from the activity indices for Th, Ra and K and expressed as the activity concentration index (ACI). Gamma spectroscopy is a non-destructive technique frequently used to simultaneously determine the indices for several radionuclides. Spectral interpretation poses a number of challenges, including identification of γ-lines subject to summing-in effects, interference from other γ-ray emitting radionuclides and the time required to reach secular equilibrium. Read More

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http://dx.doi.org/10.1016/j.apradiso.2018.09.019DOI Listing
December 2018

Thromboelastometry Identified Alteration of Clot Stabilization and Factor XIII Supplementation Need in a Patient with Decompensated Liver Disease Undergoing Liver Biopsy.

Case Rep Gastrointest Med 2018 29;2018:6360543. Epub 2018 Aug 29.

Hospital Leforte, Department of Critical Care, São Paulo, SP, Brazil.

Liver disease has been considered the prototype of hemorrhagic disease. Disorder in any component of coagulation system can lead to hemorrhage. Deficiency of factor XIII may impair clot strength and clot stabilization and can be accessed by thromboelastometry. Read More

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http://dx.doi.org/10.1155/2018/6360543DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6136510PMC
August 2018
6 Reads

Gene Variant (V34L) and Residual Circulating FXIIIA Levels Predict Short- and Long-Term Mortality in Acute Myocardial Infarction after Coronary Angioplasty.

Int J Mol Sci 2018 Sep 14;19(9). Epub 2018 Sep 14.

Centre of Haemostasis & Thrombosis, Department of Biomedical and Specialty Surgical Sciences, Section of Medical Biochemistry, Molecular Biology & Genetics, University of Ferrara, 44121 Ferrara, Italy.

Factor XIIIA (FXIIIA) levels are independent predictors of early prognosis after acute myocardial infarction (AMI) and the Valine-to-Leucine (V34L) single nucleotide polymorphism (SNP) seems associated with lower AMI risk. Since the long-term AMI prognosis merits deeper investigation, we performed an observational study evaluating relationships between FXIIIA residual levels, cardiovascular risk-factors, and inherited genetic predispositions. FXIIIA V34L was genotyped in 333 AMI patients and a five-year follow-up was performed. Read More

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http://www.mdpi.com/1422-0067/19/9/2766
Publisher Site
http://dx.doi.org/10.3390/ijms19092766DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6165350PMC
September 2018
4 Reads
2.862 Impact Factor