11,041 results match your criteria Factor IX


Clotting factor activity in fresh frozen plasma after thawing with a new radio wave thawing device.

Transfusion 2019 Mar 18. Epub 2019 Mar 18.

Department of Transfusion Medicine and Hemostaseology, University Hospital Erlangen, Friedrich-Alexander-University, Erlangen, Germany.

Background: Massive hemorrhage usually results in rapid need of blood products. Patients in need of fresh frozen plasma (FFP) might benefit from shorter thawing times using a novel radio wave device. So far, only one study on the prototype has been published. Read More

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http://dx.doi.org/10.1111/trf.15246DOI Listing

Strategies for Individualized Dosing of Clotting Factor Concentrates and Desmopressin in Hemophilia A and B.

Ther Drug Monit 2019 Apr;41(2):192-212

Department of Hospital Pharmacy-Clinical Pharmacology, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands.

Hemophilia A and hemophilia B are hereditary bleeding disorders, caused by a deficiency of clotting factor VIII or clotting factor IX, respectively. To treat and prevent bleedings, patients can administer clotting factor concentrates (hemophilia A and B) or desmopressin (hemophilia A). Both clotting factor concentrates and desmopressin are currently dosed according to the patients' body weight. Read More

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http://dx.doi.org/10.1097/FTD.0000000000000625DOI Listing

Anesthesia management of arthroscopic ankle arthrodesis for a hemophilia patient after living-donor liver transplantation.

Intractable Rare Dis Res 2019 Feb;8(1):56-59

Department of Anesthesia, Research Hospital, The Institute of Medical Science, The University of Tokyo, Tokyo, Japan.

Hemophilia is an X-linked recessive inherited coagulation disorder. We report the anesthesia management of a hemophilia patient who underwent arthroscopic ankle arthrodesis after living-donor liver transplantation due to cirrhosis. The 35-year-old male patient with hemophilia B was diagnosed with cirrhosis due to hepatitis C virus at the age of 23 years and underwent biologically-related partial liver transplantation at the age of 29 years. Read More

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http://dx.doi.org/10.5582/irdr.2018.01127DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6409122PMC
February 2019

Sustained high trough factor IX activity levels with continued use of rIX-FP in adult and paediatric patients with haemophilia B.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Center for Bleeding Disorders and Coagulation, Careggi University Hospital, Florence, Italy.

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http://dx.doi.org/10.1111/hae.13735DOI Listing

New data from the Italian National Register of Congenital Coagulopathies, 2016 Annual Survey.

Blood Transfus 2019 Feb 13. Epub 2019 Feb 13.

Department of Oncology and Molecular Medicine, Italian National Institute of Health, Rome, Italy.

Background: In Italy, the National Register of Congenital Coagulopathies (NRCC) collects epidemiological and therapeutic data from patients affected by haemophilia A (HA), haemophilia B (HB), von Willebrand's disease (vWD) and other rare coagulation disorders. Here we present data from the 2016 annual survey.

Materials And Methods: Data are provided by the Italian Haemophilia Centres, on a voluntary basis. Read More

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http://dx.doi.org/10.2450/2019.0211-18DOI Listing
February 2019
1 Read

Engineering, and production of functionally active human Furin in N. benthamiana plant: In vivo post-translational processing of target proteins by Furin in plants.

PLoS One 2019 12;14(3):e0213438. Epub 2019 Mar 12.

Akdeniz University, Department of Agricultural Biotechnology, Antalya, Turkey.

A plant expression platform with eukaryotic post-translational modification (PTM) machinery has many advantages compared to other protein expression systems. This promising technology is useful for the production of a variety of recombinant proteins including, therapeutic proteins, vaccine antigens, native additives, and industrial enzymes. However, plants lack some of the important PTMs, including furin processing, which limits this system for the production of certain mammalian complex proteins of therapeutic value. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0213438PLOS
March 2019
2 Reads

Christmas disease in a Hovawart family resembling human hemophilia B Leyden is caused by a single nucleotide deletion in a highly conserved transcription factor binding site of the F9 gene promoter.

Haematologica 2019 Mar 7. Epub 2019 Mar 7.

Institute of Genetics, University of Bern, Bern, Switzerland.

Hemophilia B is a classical monogenic X-chromosomal recessively transmitted bleeding disorder caused by genetic variants within the coagulation factor IX gene. Although hemophilia B has been described in dogs, it has not yet been reported in the Hovawart. Here we describe the identification of a Hovawart family transmitting typical signs of an X-linked bleeding disorder. Read More

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http://dx.doi.org/10.3324/haematol.2018.215426DOI Listing

Inhibitors among patients with hemophilia in Basra, Iraq - A single center experience.

Niger J Clin Pract 2019 Mar;22(3):416-421

Department of Pediatrics, College of Medicine, University of Basra; Center for Hereditary Blood Disease/Basra Health Directorate, Basra, Iraq.

Introduction: Inhibitor formation is a major complication of hemophilia treatment because it interferes with the clinical response to factor replacement and causes significant morbidity. This cross-sectional study was conducted to assess the presence and frequency of inhibitors among registered person with hemophilia and to identify risk factors associated with inhibitor development.

Patients And Methods: A total of 143 hemophilics, 118 with hemophilia A (HA) and 25 with hemophilia B (HB), were enrolled for the study. Read More

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http://dx.doi.org/10.4103/njcp.njcp_388_18DOI Listing
March 2019
1 Read

Metastatic Clear-cell Renal Cell Carcinoma With a Long-term Response to Sunitinib: A Distinct Phenotype Independently Associated With Low PD-L1 Expression.

Clin Genitourin Cancer 2019 Feb 4. Epub 2019 Feb 4.

Service d'Anatomie et Cytologie Pathologiques, Université de Rennes 1, Université Bretagne Loire, Rennes, France; Unité Mixte de Recherche 6290-Institut de Génétique et Développement de Rennes, Rennes, France.

Background: Long-term responders (LTRs) are defined by at least 18 months of response to sunitinib in metastatic clear-cell renal cell carcinoma (ccRCC). Well-described by clinical studies, the phenotype of these tumors has never been explored.

Patients And Methods: In a retrospective and multicenter study, 90 ccRCCs of patients with metastatic disease were analyzed. Read More

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http://dx.doi.org/10.1016/j.clgc.2019.01.014DOI Listing
February 2019
2 Reads

Implications of Oxalate as a Novel Risk Factor for Chronic Kidney Disease.

Authors:
Joachim H Ix

JAMA Intern Med 2019 Mar 4. Epub 2019 Mar 4.

Division of Nephrology-Hypertension, Department of Medicine, University of California, San Diego.

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http://dx.doi.org/10.1001/jamainternmed.2018.7987DOI Listing

Gold nano-urchin integrated label-free amperometric aptasensing human blood clotting factor IX: A prognosticative approach for "Royal disease".

Biosens Bioelectron 2019 Apr 19;131:128-135. Epub 2019 Feb 19.

Centre of Innovative Nanostructure & Nanodevices, Universiti Teknologi PETRONAS, 32610 Bandar Seri Iskandar, Perak Darul Ridzuan, Malaysia.

This article is clearly presenting the development of a biosensor for human factor IX (FIX) to diagnose the blood clotting deficiency, a so-called 'Royal disease' using an interdigitated electrode (IDE) with the zinc oxide surface modification. Gold nano-urchins (GNUs) with 60 nm in diameter was integrated into a streptavidin-biotinylated aptamer strategy to enhance the active surface area. Two different comparative studies have been done to validate the system to be practiced in the current work holds with a higher capability for the high-performance sense. Read More

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http://dx.doi.org/10.1016/j.bios.2019.02.006DOI Listing

A new in silico approach to investigate molecular aspects of Factor IX (F9) missense causative mutations and their impact on the Hemophilia B severity.

Hum Mutat 2019 Feb 28. Epub 2019 Feb 28.

Departamento de Genética, Instituto de Biociências, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

Factor IX (encoded by F9) is a protein in the coagulation process, where its lack or deficiency leads to hemophilia B. This condition has been much less studied than hemophilia A, especially in Latin America. We analyzed the structural and functional impact of 54 missense mutations (18 reported by us previously, and 36 other mutations from the Factor IX database) through molecular modeling approaches. Read More

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http://dx.doi.org/10.1002/humu.23733DOI Listing
February 2019

Strategies for individualized dosing of clotting factor concentrates and desmopressin in hemophilia A and B.

Ther Drug Monit 2019 Feb 26. Epub 2019 Feb 26.

Hospital Pharmacy-Clinical Pharmacology, Amsterdam UMC, University of Amsterdam, the Netherlands.

Hemophilia A and B are hereditary bleeding disorders, caused by a deficiency of clotting factor VIII or clotting factor IX, respectively. To treat and prevent bleedings, patients can administer clotting factor concentrates (hemophilia A and B) or desmopressin (hemophilia A). Both clotting factor concentrates and desmopressin are currently dosed according to the patients' body weight. Read More

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http://dx.doi.org/10.1097/FTD.0000000000000625DOI Listing
February 2019
1 Read

Factor IX inhibitors: Clinical and laboratory profiles of two patients with severe haemophilia B.

Haemophilia 2019 Feb 28. Epub 2019 Feb 28.

Nationwide Children's Hospital, Columbus, Ohio.

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http://dx.doi.org/10.1111/hae.13696DOI Listing
February 2019

Functions of the Heterologous Intron-Derived Fragments Intra and Extra Factor IX-cDNA Coding Region on the Human Factor IX Expression in HepG2 and Hek-293T Cells.

Iran J Biotechnol 2018 May 15;16(2):e1753. Epub 2018 May 15.

Department of Cellular and Molecular Biotechnology, Institute of Biotechnology, Urmia University, Urmia, Iran.

Background: Human FIX (hFIX) gene transfer into hepatocytes has provided a novel approach for treatment of hemophilia B. To obtain an improved expression of hFIX, the functional -expressing plasmids with appropriate intron-derived fragments which facilitate transcription and promote an efficient 3'-end formation of mRNAs are required.

Objectives: We aim to evaluate the functions of the heterologous intron-derived fragments intra and extra hFIX-cDNA coding region with respect to the hFIX expression in the hepatocytes and kidney cells. Read More

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http://dx.doi.org/10.21859/ijb.1753DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6371630PMC
May 2018
1 Read

Plasma kallikrein structure reveals apple domain disc rotated conformation compared to factor XI.

J Thromb Haemost 2019 Feb 23. Epub 2019 Feb 23.

Centre for Biomolecular Sciences, School of Pharmacy, University of Nottingham, Nottingham, UK.

Essentials Zymogen PK is activated to PKa and cleaves substrates kininogen and FXII contributing to bradykinin generation. Monomeric PKa and dimeric homologue FXI utilize the N-terminal apple domains to recruit substrates. A high-resolution 1. Read More

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http://dx.doi.org/10.1111/jth.14418DOI Listing
February 2019

Direct-Acting Oral Anticoagulants and Warfarin-Associated Intracerebral Hemorrhage Protocol Reduces Timing of Door to Correction Interventions.

J Neurosci Nurs 2019 Apr;51(2):89-94

Questions or comments about this article may be directed to Rosemary C. Olivier, MSN RN CCRN SCRN, at She is a Critical Care Nurse Leader, St Jude Medical Center, Fullerton, CA. Diane Gleeson, MSN ANP-BC, is Neurology Nurse Practitioner, St Jude Medical Center, Fullerton, CA. Claudia Skinner, DNP RN CIC CCRN-K NE-BC FAPIC, is Director, Center of Excellence, St Jude Medical Center, Fullerton, CA. Marysol Cacciata, MSN RN CCRN-K, is Research Coordinator, St Jude Medical Center, Fullerton, CA. Mary Wickman, PhD RN, is Professor and Director, Nursing, Vanguard University, Costa Mesa, CA.

Background: Intracerebral hemorrhage (ICH) is a life-threatening complication of oral anticoagulant therapy that sometimes results in hematoma expansion after onset. Our facility did not have a standardized process for treating oral anticoagulant-associated ICH; this resulted in lag times from order to reversal agent administration.

Purpose: The aim of this study was to examine the impact of a rapid anticoagulant reversal protocol, combined with warfarin and direct-acting oral anticoagulant therapy, in decreasing door to first intervention times. Read More

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http://dx.doi.org/10.1097/JNN.0000000000000430DOI Listing
April 2019
4 Reads

Factor IX assays in treated hemophilia B patients.

Ann Biol Clin (Paris) 2018 Feb;77(1):41-52

Service d'hématologie hémostase, Hospices civils de Lyon, Bron, France.

Replacement therapy with plasma-derived or recombinant FIX (pdFIX or rFIX) concentrates is the standard of treatment in patients with hemophilia B. The method predominantly used for measuring factor IX (FIX:C) levels is the one-stage clotting assay (OSA) but this method depends on the activated partial thromboplastin time (APTT) reagent and the coagulation analyzer used, and wide variations in the measurements of FIX recovery have been reported with some factor concentrates. The French study group on the biology of hemorrhagic diseases (a collaborative group of the GFHT and MHEMO network), presents a review of the literature and proposals for the monitoring of FIX:C levels in treated hemophilia B patients. Read More

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http://dx.doi.org/10.1684/abc.2019.1414DOI Listing
February 2018
1 Read

Bilateral subthalmic nucleus deep brain stimulation with microelectrode recordings in the setting of mild inherited Hemophilia B: A case report.

Int J Neurosci 2019 Feb 22:1-4. Epub 2019 Feb 22.

c Department of Neurosurgery , University of Kansas Medical Center , 3901 Rainbow Boulevard, MS 3021 , Kansas City , KS 66160.

Hemophilia B is an X linked recessive deficiency of factor IX that presents with a range of clinical severity that co-relates with factor levels. Although guidelines exist to guide perioperative hemostasis in such patients, there is scarce data on elective high-risk neurosurgeries, resulting in a reluctance to offer these patients elective neurosurgeries. These patients thus rarely if ever undergo such procedures. Read More

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http://dx.doi.org/10.1080/00207454.2019.1586685DOI Listing
February 2019

The angiotensin-(1-7)/Mas receptor axis protects from endothelial cell senescence via klotho and Nrf2 activation.

Aging Cell 2019 Feb 17:e12913. Epub 2019 Feb 17.

Department of Pharmacology, Faculty of Medicine, Universidad Autónoma de Madrid, Madrid, Spain.

Endothelial cell senescence is a hallmark of vascular aging that predisposes to vascular disease. We aimed to explore the capacity of the renin-angiotensin system (RAS) heptapeptide angiotensin (Ang)-(1-7) to counteract human endothelial cell senescence and to identify intracellular pathways mediating its potential protective action. In human umbilical vein endothelial cell (HUVEC) cultures, Ang II promoted cell senescence, as revealed by the enhancement in senescence-associated galactosidase (SA-β-gal+) positive staining, total and telomeric DNA damage, adhesion molecule expression, and human mononuclear adhesion to HUVEC monolayers. Read More

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http://doi.wiley.com/10.1111/acel.12913
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http://dx.doi.org/10.1111/acel.12913DOI Listing
February 2019
4 Reads

Revealing membrane alteration in cellsoverexpressing CA IX and EGFR by Surface-Enhanced Raman Scattering.

Sci Rep 2019 Feb 12;9(1):1832. Epub 2019 Feb 12.

Department of Physics E. Pancini, University of Naples Federico II, Complesso Univesitario Monte S. Angelo, Via Cintia, I-80126, Naples, Italy.

Sensitive detection of altered proteins expression in plasma membranes is of fundamental importance, for both diagnostic and prognostic purposes. Surface-Enhanced Raman Scattering (SERS) has proven to be a quite sensitive approach to detect proteins, even in very diluted samples. However, proteins detection in complex environment, such as the cellular membrane, is still a challenge. Read More

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http://dx.doi.org/10.1038/s41598-018-37997-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6372785PMC
February 2019

BML-111 accelerates the resolution of inflammation by modulating the Nrf2/HO-1 and NF-κB pathways in rats with ventilator-induced lung injury.

Int Immunopharmacol 2019 Apr 10;69:289-298. Epub 2019 Feb 10.

Department of Critical Care Medicine, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China; Institute of Anesthesiology and Critical Care Medicine, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China. Electronic address:

The timely resolution of pulmonary inflammation coordinated by endogenous pro-resolving mediators helps limit lung tissue injury, but few endogenous pro-resolving mediators that are normally operative during acute inflammation. The protective effects of BML-111 (5(S)-6(R)-7-trihydroxyheptanoic acid methyl ester), a potent commercially available anti-inflammatory and pro-resolving mediator, on ventilation-induced lung injury (VILI) have been extensively studied, but its characteristics as a pro-resolving mediator have not. Here, anesthetized Sprague-Dawley rats were ventilated with a high tidal volume (20 mL/kg, HV) for 1 h and randomly allocated to recover for 6, 12, 24, 48, 72, 96 or 168 h; BML-111 was administered at the peak of inflammation to evaluate its pro-resolving effect on VILI. Read More

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http://dx.doi.org/10.1016/j.intimp.2019.02.005DOI Listing
April 2019
2 Reads

Carbonic anhydrase-IX inhibition enhances the efficacy of hexokinase II inhibitor for hepatocellular carcinoma in a murine model.

J Bioenerg Biomembr 2019 Feb 12. Epub 2019 Feb 12.

Department of Internal Medicine and Liver Research Institute, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, Republic of Korea.

Hypoxic conditions, which large or infiltrative hypovascular tumors may encounter, also produce acidic environments. Carbonic anhydrase-IX (CA-IX), an enzyme involved in lowering pH, is overexpressed in hepatocellular carcinoma (HCC). In the present study, whether inhibition of CA-IX enhances the efficacy of a hexokinase II inhibitor in an in vivo murine model was examined and its prognostic implication in HCC patients was investigated. Read More

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http://dx.doi.org/10.1007/s10863-019-09788-6DOI Listing
February 2019
2 Reads
3.212 Impact Factor

First line treatment of adult patients with primary immune thrombocytopenia: a real-world study.

Platelets 2019 Feb 11:1-7. Epub 2019 Feb 11.

a Department of Hematology , Qilu Hospital, Shandong University , Jinan , China.

Immune thrombocytopenia (ITP) is an autoimmune disease with a mild to severe risk of bleeding complications. First line treatment includes corticosteroids, immunoglobulins, or other. In this large cohort study, first-line strategies for treatment-naive adult primary ITP was studied in a real-world setting. Read More

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http://dx.doi.org/10.1080/09537104.2019.1572875DOI Listing
February 2019
3 Reads

Establishment of the WHO 2nd International Standard Factor V, plasma (16/374): communication from the SSC of the ISTH.

J Thromb Haemost 2019 Feb 7. Epub 2019 Feb 7.

National Institute for Biological Standards and Control, Potters Bar, Hertfordshire, UK.

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http://dx.doi.org/10.1111/jth.14403DOI Listing
February 2019
2 Reads

A Nonsense Mitochondrial DNA Mutation Associates with Dysfunction of HIF1 in a Von Hippel-Lindau Renal Oncocytoma.

Oxid Med Cell Longev 2019 9;2019:8069583. Epub 2019 Jan 9.

Department of Medical and Surgical Sciences (DIMEC), Unit of Medical Genetics, University of Bologna Medical School, Bologna 40138, Italy.

The Von Hippel-Lindau (VHL) syndrome has been rarely associated with renal oncocytomas, and tumors usually show HIF1 chronic stabilization. By contrast, oncocytomas mainly associated with respiratory chain (RC) defects due to severe mitochondrial DNA (mtDNA) mutations are incapable of stabilizing HIF1, since oxygen consumption by the RC is dramatically diminished and prolylhydroxylase activity is increased by -ketoglutarate accumulation following Krebs cycle slowdown. Here, we investigate the cooccurrence of a pseudohypoxic condition with oncocytic transformation in a case of VHL-associated renal oncocytoma. Read More

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http://dx.doi.org/10.1155/2019/8069583DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343171PMC
March 2019
3 Reads

Sodium-site in serine protease domain of human coagulation factor IXa: evidence from the crystal structure and molecular dynamics simulations study.

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

Department of Orthopaedic Surgery, University of California, Los Angeles, CA, USA.

Essentials Consensus sequence and biochemical data suggest a Na -site in the factor (F) IXa protease domain. X-ray structure of the FIXa EGF2/protease domain at 1.37 Å reveals a Na -site not observed earlier. Read More

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http://dx.doi.org/10.1111/jth.14401DOI Listing
February 2019
1 Read

Gold-nanorod enhances dielectric voltammetry detection of c-reactive protein: A predictive strategy for cardiac failure.

Biosens Bioelectron 2019 Apr 29;130:40-47. Epub 2019 Jan 29.

Institute of Nano Electronic Engineering, Universiti Malaysia Perlis, 01000 Kangar, Perlis, Malaysia; School of Bioprocess Engineering, Universiti Malaysia Perlis, 02600 Arau, Perlis, Malaysia. Electronic address:

This paper primarily demonstrates the approach to enhance the sensing performance on antigen C-reactive protein (CRP) and anti-CRP antibody binding event. A nanogapped electrode structure with the gap of ~100 nm was modified by the anti-CRP antibody (Probe) to capture the available CRP. In order to increase the amount of antigen to be captured, a gold nanorod with 119 nm in length and 25 nm in width was integrated, to increase the surface area. Read More

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http://dx.doi.org/10.1016/j.bios.2019.01.042DOI Listing
April 2019
1 Read

Protein-Engineered Coagulation Factors for Hemophilia Gene Therapy.

Mol Ther Methods Clin Dev 2019 Mar 31;12:184-201. Epub 2018 Dec 31.

The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Hemophilia A (HA) and hemophilia B (HB) are X-linked bleeding disorders due to inheritable deficiencies in either coagulation factor VIII (FVIII) or factor IX (FIX), respectively. Recently, gene therapy clinical trials with adeno-associated virus (AAV) vectors and protein-engineered transgenes, B-domain deleted (BDD) FVIII and FIX-Padua, have reported near-phenotypic cures in subjects with HA and HB, respectively. Here, we review the biology and the clinical development of FVIII-BDD and FIX-Padua as transgenes. Read More

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http://dx.doi.org/10.1016/j.omtm.2018.12.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6349562PMC
March 2019
1 Read

Velvet Antler Mobilizes Endothelial Progenitor Cells to Promote Angiogenesis and Repair Vascular Endothelial Injury in Rats Following Myocardial Infarction.

Front Physiol 2018 17;9:1940. Epub 2019 Jan 17.

Department of Integrative Cardiology, China-Japan Friendship Hospital, Beijing, China.

This investigation examined the effect of velvet antler (VA) on endothelial progenitor cells (EPCs) and the associated effects to promote angiogenesis and repair vascular endothelial injury in rats with myocardial infarction (MI). VA was analyzed by liquid chromatography-mass spectrometry. Male Sprague Dawley rats were randomly divided into four groups: sham, MI, VA, and VA + DAPT (gamma-secretase inhibitor IX, a specific blocker of the Notch signaling pathway) group. Read More

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http://dx.doi.org/10.3389/fphys.2018.01940DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6344410PMC
January 2019
3 Reads

Models and Studies of Aging: Executive Summary of a Report from the U13 Conference Series.

J Am Geriatr Soc 2019 Mar 29;67(3):428-433. Epub 2019 Jan 29.

University of Michigan, Ann Arbor, Michigan.

The American Geriatrics Society convened a conference in Bethesda, Maryland, to explore models and studies of aging. This was the second of three conferences, supported by a U13 grant from the National Institute on Aging, to aid recipients of Grants for Early Medical/Surgical Specialists Transition to Aging Research (GEMSSTAR) in integrating geriatrics into their specialties. Recognizing that aging is the largest risk factor for multiple chronic diseases and age-related loss of resilience, the conference organizers focused scientific sessions on how targeting age-related mechanisms can delay, prevent, or reverse geriatric syndromes, age-related chronic diseases, and loss of resilience. Read More

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http://dx.doi.org/10.1111/jgs.15788DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6403012PMC
March 2019
2 Reads

Ferroptosis as a target for protection against cardiomyopathy.

Proc Natl Acad Sci U S A 2019 Feb 28;116(7):2672-2680. Epub 2019 Jan 28.

The First Affiliated Hospital, School of Public Health, Institute of Translational Medicine, Zhejiang University School of Medicine, 310058 Hangzhou, China;

Heart disease is the leading cause of death worldwide. A key pathogenic factor in the development of lethal heart failure is loss of terminally differentiated cardiomyocytes. However, mechanisms of cardiomyocyte death remain unclear. Read More

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http://dx.doi.org/10.1073/pnas.1821022116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6377499PMC
February 2019
2 Reads

Fluorine-18 click radiosynthesis and microPET/CT evaluation of a small peptide-a potential PET probe for carbonic anhydrase IX.

Bioorg Med Chem 2019 Mar 17;27(5):785-789. Epub 2019 Jan 17.

Shanghai Institute of Applied Physics, Chinese Academy of Sciences, Shanghai 201800, China. Electronic address:

Carbonic anhydrase IX (CA IX) is the first carbonic anhydrase found to be associated with cancer that is over-expressed in a variety of human solid tumors. As a surrogate marker for hypoxia, the expression of CA IX is strongly upregulated in hypoxic tumors by hypoxia and hypoxia-inducible factor 1a (HIF-1a). In our pursuit of a CA IX-specific PET probe, we designed and synthesized a peptide-based CA IX imaging probe by the efficient click reaction of 1,3-dipolar cycloaddition of terminal alkynes and organic azides. Read More

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http://dx.doi.org/10.1016/j.bmc.2019.01.014DOI Listing
March 2019
1 Read

Safety of recombinant coagulation factors in treating hemophilia.

Expert Opin Drug Saf 2019 Feb 22;18(2):75-85. Epub 2019 Feb 22.

b Department of Hematology , University of Florence , Italy.

Introduction: During the last decade, new FVIII/IX concentrates have been developed for the treatment of patients affected by hemophilia A/B. Significant progress has been achieved regarding their half-life, but the old issue of immunogenicity and new concerns about safety need to be addressed. Areas covered: After the implementation of virucidal methods, both plasma-derived and recombinant clotting factor concentrates achieved a very safe profile. Read More

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http://dx.doi.org/10.1080/14740338.2019.1574743DOI Listing
February 2019

Production of recombinant human factor IX by propeptide modification in Drosophila S2 cell line.

Biotechnol Lett 2019 Mar 23;41(3):347-355. Epub 2019 Jan 23.

Department of Molecular Medicine, Institute of Medical Biotechnology, National Institute of Genetic Engineering and Biotechnology, P.O. Box: 14965/161, Tehran, Iran.

Objective: To compare the effect of pre-propeptide (pre-pro) of the human prothrombin (hPT), with both the native and an R-9N mutant forms of the human factor IX (hFIX) pre-pro on the hFIX carboxylation, in Drosophila cell.

Results: The three different pre-pro sequences, equipped with Drosophila Kozak, were joined to the mature hFIX cDNA and were subjected to transient expression analysis of hFIX in the S2 Drosophila cells, compared to that of a native hFIX cDNA, with its native Kozak. Replacement of the hFIX pre-pro sequence with that of hPT increased the biological activity of hFIX, significantly. Read More

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http://dx.doi.org/10.1007/s10529-019-02643-zDOI Listing
March 2019
1 Read

Laboratory assay measurement of modified clotting factor concentrates: a review of the literature and recommendations for practice.

J Thromb Haemost 2019 Jan 22. Epub 2019 Jan 22.

Cambridge Haemophilia and Thrombophilia Centre, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

Over the past several years, novel modified clotting factor concentrates (CFCs) have been introduced into practice and are now widely prescribed in the countries where they are licensed. These products allow for less frequent infusions of CFC, thereby providing improved convenience and/or higher trough levels. They have been extensively studied for prophylaxis, episodic treatment of bleeding and for surgical prophylaxis. Read More

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http://dx.doi.org/10.1111/jth.14394DOI Listing
January 2019
2 Reads

FIXing postinfusion monitoring: Assay experiences with N9-GP (nonacog beta pegol; Refixia ; Rebinyn ).

Haemophilia 2019 Jan;25(1):154-161

Novo Nordisk A/S, Måløv, Denmark.

N9-GP (nonacog beta pegol; Refixia ; Rebinyn , Novo Nordisk A/S, Bagsvaerd, Denmark) is a glycoPEGylated extended half-life recombinant factor IX (rFIX) that exhibits efficacy and potency comparable to unmodified FIX molecules in non-clinical models. Phase 3 clinical trials have confirmed the efficacy and tolerability of N9-GP for the prevention and on-demand treatment of bleeding episodes in patients with haemophilia B. Recent studies have shown that PEGylation affects clotting times in activated partial thromboplastin time (aPTT)-based one-stage activity assays due to interaction between the FIX molecule and certain aPTT reagents. Read More

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http://dx.doi.org/10.1111/hae.13671DOI Listing
January 2019
4 Reads

Walking the Walk: Testing a Theory-Derived Measure of Campus Sexual Assault Policy Implementation.

J Interpers Violence 2019 Jan 18:886260518824649. Epub 2019 Jan 18.

2 Stony Brook University, NY, USA.

Institutional Theory posits that organizations sometimes engage in practices that are inconsistent with their stated policies-a process called "decoupling"-due to three types of legitimacy pressures: normative, coercive, and mimetic. Limited previous research suggests that such decoupling between policy and practice may be occurring in institutions of higher education (IHEs) when addressing sexual assault. The objective of this study was to test a measure designed to assess sexual assault policy implementation using the constructs of Institutional Theory. Read More

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http://dx.doi.org/10.1177/0886260518824649DOI Listing
January 2019
2 Reads

[Gene therapy of haemophilia - has the cure come within reach?]

Postepy Biochem 2018 12;64(4):318-322

Instytut Immunologii i Terapii Doświadczalnej im. Ludwika Hirszfelda PAN, Wrocław.

Haemophilia is a bleeding disorder (usually congenital) caused by the deficiency of coagulation factor VIII (haemophilia A) or IX (haemophilia B). The genes encoding factors VIII and IX are located on the X chromosome, so the symptoms of congenital haemophilia A and B occur predominantly in males. Recurring episodes of spontaneous bleeding into joints are the main symptom of haemophilia, which lead to haemophilic artropathy. Read More

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http://dx.doi.org/10.18388/pb.2018_145DOI Listing
December 2018
9 Reads

Extending recombinant factor IX Fc fusion protein dosing interval to 14 or more days in patients with hemophilia B.

Res Pract Thromb Haemost 2019 Jan 29;3(1):109-113. Epub 2018 Nov 29.

Sobi Stockholm Sweden.

Background: In the phase 3 B-LONG study (NCT01027364), prophylaxis with recombinant factor IX Fc fusion protein (rFIXFc) every 7 to >14 days was associated with low annualized bleed rates (ABRs) in males aged ≥12 years with severe hemophilia B. The long-term safety and efficacy of rFIXFc prophylaxis was confirmed in the B-YOND study (NCT01425723), an extension of the B-LONG clinical trial.

Objective: The aim of this post-hoc analysis was to evaluate the efficacy of a ≥14-day rFIXFc dosing interval in patients treated prophylactically during B-LONG or B-YOND. Read More

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http://dx.doi.org/10.1002/rth2.12163DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332734PMC
January 2019
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Investigation of the Prognostic Role of Carbonic Anhydrase 9 (CAIX) of the Cellular mRNA/Protein Level or Soluble CAIX Protein in Patients with Oral Squamous Cell Carcinoma.

Int J Mol Sci 2019 Jan 16;20(2). Epub 2019 Jan 16.

Department of Oral and Maxillofacial Plastic Surgery, Martin Luther University Halle-Wittenberg, 06120 Halle (Saale), Germany.

s Carbonic anhydrase 9 (CAIX) is an important protein that stabilizes the extracellular pH value and is transcriptionally regulated by hypoxia-inducible factor 1 (HIF1), but more stable than HIF1α. Here we show a comparative study that examines the prognostic value of CA9 mRNA, CAIX protein of tumor cells and secreted CAIX protein for oral squamous cell carcinoma (OSCC) patients. Tumor samples from 72 OSCC patients and 24 samples of normal tissue were analyzed for CA9 mRNA levels. Read More

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http://www.mdpi.com/1422-0067/20/2/375
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http://dx.doi.org/10.3390/ijms20020375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6359351PMC
January 2019
14 Reads

Carvacrol Depends on Heme Oxygenase-1 (HO-1) to Exert Antioxidant, Anti-inflammatory, and Mitochondria-Related Protection in the Human Neuroblastoma SH-SY5Y Cells Line Exposed to Hydrogen Peroxide.

Neurochem Res 2019 Jan 16. Epub 2019 Jan 16.

Grupo de Estudos em Neuroquímica e Neurobiologia de Moléculas Bioativas, Universidade Federal de Mato Grosso (UFMT), Av. Fernando Corrêa da Costa, 2367, Cuiaba, MT, CEP 78060-900, Brazil.

The link between mitochondrial dysfunction, redox impairment, and inflammation leads to increased rates of brain cells loss in neurodegenerative diseases and in affective disorders. Carvacrol (CAR) is a component of essential oils found in Labiatae. CAR exerts antioxidant and anti-inflammatory effects in different cell types, as assessed in both in vitro and in vivo experimental designs. Read More

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http://dx.doi.org/10.1007/s11064-019-02724-5DOI Listing
January 2019
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Deletion of the Riemerella anatipestifer type IX secretion system gene sprA results in differential expression of outer membrane proteins and virulence.

Avian Pathol 2019 Jan 14:1-13. Epub 2019 Jan 14.

a State Key Laboratory of Agricultural Microbiology, College of Veterinary Medicine , Huazhong Agricultural University , Wuhan , People's Republic of China.

Riemerella anatipestifer (RA), the causative agent of infectious serositis that targets ducklings and other poultry, secretes protein via the type IX secretion system (T9SS). The proteins transported by T9SS are located on the bacterial cell surface or secreted into the extracellular milieu. In this study, a sprA deletion mutant was constructed encoding a core protein of T9SS to investigate its influence on outer membrane protein expression and its role in virulence. Read More

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http://dx.doi.org/10.1080/03079457.2019.1566594DOI Listing
January 2019
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Ramsay Hunt syndrome.

J Dent Anesth Pain Med 2018 Dec 28;18(6):333-337. Epub 2018 Dec 28.

Department of Anesthesiology and Pain Medicine, School of Dentistry, Kyungpook National University, Daegu, Republic of Korea.

Ramsay Hunt syndrome is a type of acute herpes zoster, which occurs by reactivation of the varicella-zoster virus at the geniculate ganglion. Clinical presentation of Ramsay Hunt syndrome includes a vesicular rash on the ear (herpes zoster oticus) or in the oral mucosa accompanied by acute peripheral facial nerve paralysis. Other cranial nerves such as V, IX, XI, and XII are often involved. Read More

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http://dx.doi.org/10.17245/jdapm.2018.18.6.333DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323042PMC
December 2018
14 Reads

How to Optimize Activated Partial Thromboplastin Time (APTT) Testing: Solutions to Establishing and Verifying Normal Reference Intervals and Assessing APTT Reagents for Sensitivity to Heparin, Lupus Anticoagulant, and Clotting Factors.

Semin Thromb Hemost 2019 Feb 10;45(1):22-35. Epub 2019 Jan 10.

Department of Haematology, NSW Health Pathology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.

The activated partial thromboplastin time (APTT) assay is a very common coagulation test, used for several reasons. The test is conventionally used for assessing the contact factor (intrinsic) pathway of blood coagulation, and thus for screening deficiencies in this pathway, most typically factors VIII, IX, and XI. The APTT is also sensitive to contact factor deficiencies, including factor XII, prekallikrein, and high-molecular-weight kininogen. Read More

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http://dx.doi.org/10.1055/s-0038-1677018DOI Listing
February 2019
6 Reads

Initiation of Pediatric Clinical Trials for Coagulation Factors: Application of Pharmacokinetics and Allometry to First-in-Pediatric Dose Selection.

Authors:
Iftekhar Mahmood

J Clin Pharmacol 2019 Jan 9. Epub 2019 Jan 9.

Office of Tissue & Advanced Therapies (OTAT), Center for Biologics Evaluation and Research, Food & Drug Administration, Silver Spring, MD, USA.

Allometric scaling is a method that can be used for the extrapolation of pharmacokinetic parameters from adults to children. Subsequently, these allometrically predicted PK parameters can be used to project a suitable dose to initiate a pediatric clinical trial. The objective of this study was to predict clearance and in vivo recovery of coagulation factors VIII and IX allometrically in children from 1 year of age to adolescents and then project the first-in-children dose. Read More

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http://dx.doi.org/10.1002/jcph.1371DOI Listing
January 2019
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Exploits Macrophage Heme Oxygenase-1 To Neutralize Oxidative Burst and TLR Signaling-Dependent Host Defense.

J Immunol 2019 Feb 28;202(3):827-840. Epub 2018 Dec 28.

Department of Biochemistry, University of Calcutta, Kolkata 700019, India;

Suppression of host oxidative burst is essential for survival of the intracellular parasite Screening of macrophage antioxidant enzymes during infection revealed marked upregulation of the heme-degrading enzyme, heme oxygenase-1 (HO-1). Moreover, HO-1-silenced RAW macrophages depicted increased superoxide production and decreased parasite survival. HO-1 induction decreased cellular heme content, thereby inhibiting the heme-dependent maturation of gp91phox, a catalytic component of major reactive oxygen species-producing enzyme NAD(P)H oxidase. Read More

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http://www.jimmunol.org/lookup/doi/10.4049/jimmunol.1800958
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http://dx.doi.org/10.4049/jimmunol.1800958DOI Listing
February 2019
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4.922 Impact Factor

[Effects of 37 centigrade volume resuscitation on coagulation function and blood lactic acid in neonates with septic shock].

Zhonghua Wei Zhong Bing Ji Jiu Yi Xue 2018 Dec;30(12):1146-1149

Department of Pediatrics, First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, Jiangxi, China (Zhong XM, Zhong M, Luo KY, Liao HQ, Wang HB); Department of Laboratory, First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, Jiangxi, China (Shen ZM); Department of Pediatrics, People's Hospital of Huichang County, Ganzhou 341000, Jiangxi, China (Tu Q). Corresponding author: Zhong Xiaoming, Email:

Objective: To investigate the effect of volume resuscitation with normal saline (NS) at 37 centigrade on the coagulation function and microcirculation of neonates with septic shock.

Methods: Children with septic shock admitted to neonatal intensive care unit (NICU) of the First Affiliated Hospital of Gannan Medical University were enrolled. Twenty-four newborns with septic shock were divided into two groups by random number table method (12 in each group), and were resuscitated with 10 mL/kg at 25 centigrade NS and 37 centigrade NS respectively on the basis of routine treatment. Read More

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http://dx.doi.org/10.3760/cma.j.issn.2095-4352.2018.012.009DOI Listing
December 2018
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Data on characterization of metalloporphyrin-mediated HO-1 and DAF induction in rat glomeruli and podocytes.

Data Brief 2019 Feb 26;22:279-285. Epub 2018 Nov 26.

Veterans Affairs Medical Center, Salem VA and Virginia Tech. Carilion School of Medicine, Roanoke, VA 24153, USA.

The data presented pertain to a research article titled "Heme Oxygenase 1 Up-Regulates Glomerular Decay Accelerating Factor Expression and Minimizes Complement Deposition and Injury" (Detsika et al., 2016). The present work provides additional data on induction and immunolocalization of heme oxygenase (HO)-1 (an antioxidant enzyme) and decay-accelerating factor (DAF) (a complement activation inhibitor) in isolated rat glomeruli and in glomerular epithelial cells (podocytes) in response to Iron Protoporphyrin IX (FePP, heme), and to non-iron protoporphyrins (PPs) with varying metal functionalities (ZnPP, SnPP), including a metal-devoid PP. Read More

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http://dx.doi.org/10.1016/j.dib.2018.11.108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305689PMC
February 2019

Whole cigarette smoke condensates induce ferroptosis in human bronchial epithelial cells.

Toxicol Lett 2019 Mar 21;303:55-66. Epub 2018 Dec 21.

Seoul Center, Korea Basic Science Institute, Seoul, Republic of Korea.

Cigarette smoke is responsible for many fatal pulmonary diseases, however, the toxic mechanism is still unclear. In this study, we first confirmed that whole cigarette smoke condensates (WCSC) contain hydrophilic elements, lipophilic and gaseous components. Then, we treated BEAS-2B cells, a normal human bronchial epithelial cell line, at dosages of 0. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03784274183175
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http://dx.doi.org/10.1016/j.toxlet.2018.12.007DOI Listing
March 2019
12 Reads