10,985 results match your criteria Factor IX


Sirtilins - the new old members of the vitamin K-dependent coagulation factor family.

J Thromb Haemost 2019 Jan 15. Epub 2019 Jan 15.

Department of Biosciences, University of Salzburg, Billrothstr. 11, A-5020, Salzburg, Austria.

Background: Vitamin K (VK)-dependent proteases are major players in blood coagulation including the initiation as well as the regulation of the cascade. Five different members of this protease family have been described comprising the coagulation factors VII (FVII), IX (FIX), X (FX), protein C (PC) and prothrombin (FII). FVII, FIX, FX and PC share a typical domain architecture with a N-terminal Gla (γ-carboxyglutamate)-domain, two EGF (epidermal growth factor-like)-domains and a C-terminal trypsin-like serine protease (SP)-domain. Read More

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http://dx.doi.org/10.1111/jth.14384DOI Listing
January 2019

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 14:1-217. Epub 2019 Jan 14.

a State Key Laboratory of Agricultural Microbiology , College of Veterinary Medicine, Huazhong Agricultural University , Wuhan , Hubei , 430070 , 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 role in virulence. Read More

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

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

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 Jan 10. 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
January 2019

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

Exploits Macrophage Heme Oxygenase-1 To Neutralize Oxidative Burst and TLR Signaling-Dependent Host Defense.

J Immunol 2018 Dec 28. 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
December 2018
4 Reads
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

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 2018 Dec 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
December 2018
3 Reads

Crosstalk Between Glucocorticoid Receptor and Early-growth Response Protein 1 Accounts for Repression of Brain-derived Neurotrophic Factor Transcript 4 Expression.

Neuroscience 2018 Dec 19;399:12-27. Epub 2018 Dec 19.

Inserm 1185, Fac Med Paris Sud, Université Paris-Saclay, Le Kremlin-Bicêtre, France. Electronic address:

The brain-derived neurotrophic factor (BDNF) is a key player in brain functions such as synaptic plasticity, stress, and behavior. Its gene structure in rodents contains 8 untranslated exons (I to VIII) whose expression is finely regulated and which spliced onto a common and unique translated exon IX. Altered Bdnf expression is associated with many pathologies such as depression, Alzheimer's disease and addiction. Read More

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http://dx.doi.org/10.1016/j.neuroscience.2018.12.012DOI Listing
December 2018
1 Read

Eptacog beta: a novel recombinant human factor VIIa for the treatment of hemophilia A and B with inhibitors.

Expert Rev Hematol 2018 Dec 21. Epub 2018 Dec 21.

a Haemophilia Treatment Centre, University Hospital Montpellier , France .

Introduction: Hemophilia A and B are X-linked recessive disorders caused by the deficiency of factor VIII or factor IX, respectively. Bleeding episodes are treated with factor replacement therapy. The most serious complication of this treatment is the development of inhibitors. Read More

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http://dx.doi.org/10.1080/17474086.2019.1560259DOI Listing
December 2018
1 Read

Oral surgery for combined haemophilia A and B. The dos and don'ts presented in a clinical scenario.

Ann Ital Chir 2018 ;89:374-378

Background: Haemophilia A (factor VIII deficiency), B (factor IX deficiency) and C (factor XI deficiency) are common genetic bleeding disorders. Most often they are caused by the absence or defective function of coagulation factors, causing inefficient blood clots.

Case Report: The present manuscript describes a rare case of a combined haemophilia A and B patient, who underwent several extractions. Read More

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January 2018
2 Reads

Continuous infusion of recombinant activated factor VII: a review of data in congenital hemophilia with inhibitors and congenital factor VII deficiency.

J Blood Med 2018 29;9:227-239. Epub 2018 Nov 29.

Clinical, Medical and Regulatory Affairs, Novo Nordisk Inc., Plainsboro, NJ, USA.

Introduction: Continuous infusion (CI) of clotting factors as a replacement therapy for perioperative hemostatic protection has been performed for many years, including with factors VIII and IX and recombinant activated factor VII (rFVIIa). This approach provides steady factor levels without requiring frequent administration of bolus doses.

Aim: To review safety, efficacy, and dosing data regarding CI of rFVIIa for hemostatic management of patients with congenital hemophilia with inhibitors (CHwI) or congenital factor VII deficiency (C7D). Read More

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http://dx.doi.org/10.2147/JBM.S184040DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276614PMC
November 2018
1 Read

Bone morphogenetic protein 4 (BMP4) loss-of-function variant associated with autosomal dominant Stickler syndrome and renal dysplasia.

Eur J Hum Genet 2018 Dec 19. Epub 2018 Dec 19.

School of Clinical Medicine, University of Cambridge, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 0SP, UK.

Stickler syndrome is a genetic disorder that can lead to joint problems, hearing difficulties and retinal detachment. Genes encoding collagen types II, IX and XI are usually responsible, but some families have no causal variant identified. We investigate a variant in the gene encoding growth factor BMP4 in a family with Stickler syndrome with associated renal dysplasia. Read More

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http://www.nature.com/articles/s41431-018-0316-y
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http://dx.doi.org/10.1038/s41431-018-0316-yDOI Listing
December 2018
6 Reads

Group B Serotypes in Pregnant Women From the Western Cape Region of South Africa.

Front Public Health 2018 4;6:356. Epub 2018 Dec 4.

Maternal Endogenous Infections Studies (MEnIS) Research Laboratories, Department of Medical Biosciences, University of the Western Cape, Bellville, South Africa.

Maternal colonization of Group B streptococci (GBS) during pregnancy is an important risk factor for neonatal morbidity and mortality. The aim of this study was to determine the prevalence and serotype distribution of GBS isolated from a cohort of pregnant women in the Western Cape, South Africa. Two ano-vaginal swabs were collected from 301 women at 28-37 weeks of gestation. Read More

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http://dx.doi.org/10.3389/fpubh.2018.00356DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6288474PMC
December 2018

Treatment of rare factor deficiencies other than hemophilia.

Blood 2018 Dec 17. Epub 2018 Dec 17.

Universita degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy

The deficiency of fibrinogen, prothrombin, factor V, VII, VIII, IX, X, XI and XIII, called rare coagulation disorders (RCDs), may result in coagulopathies leading to spontaneous or post-trauma and post-surgery hemorrhages. RCDs are characterized by a wide variety of symptoms, from mild to severe, which can vary significantly from one disease to another and from one patient to another. The most typical symptoms of all RCDs are mucosal bleedings and bleeding at the time of invasive procedures, while other life-threatening symptoms such as central nervous system bleeding and hemarthroses are mostly present only in some deficiencies (afibrinogenemia, FX and FXIII). Read More

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http://dx.doi.org/10.1182/blood-2018-06-820738DOI Listing
December 2018
1 Read

Update on clinical gene therapy for hemophilia.

Blood 2018 Dec 17. Epub 2018 Dec 17.

Wells Center for Pediatric Research, Indiana University, Indianapolis, IN, United States.

In contrast to other diverse therapies for the X-linked bleeding disorder hemophilia that are currently in clinical development, gene therapy holds the promise of a lasting cure with a single drug administration. Near to complete correction of hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) has now been achieved in patients by hepatic in vivo gene transfer. Adeno-associated viral (AAV) vectors with different viral capsids and engineered to express high-levels and in some cases hyperactive coagulation factors were employed. Read More

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http://dx.doi.org/10.1182/blood-2018-07-820720DOI Listing
December 2018
1 Read

A Study of Recombinant Factor IX in Drosophila Insect S2 Cell Lines Through Transient Gene Expression Technology.

Avicenna J Med Biotechnol 2018 Oct-Dec;10(4):265-268

Department of Biotechnology, Sabzevar Branch, Islamic Azad University, Sabzevar, Iran.

Background: Since the mass production of recombinant proteins requires the development of stable cell lines which is a time-consuming complex process, the use of transient expression on a large scale can be a comparatively useful alternative. Although various cell lines have been used for the expression of recombinant proteins, only a limited number of cells enjoy a high transfection characteristic and the ability to adapt to serum-free suspension culture easily. In the present study, the S2 cells from Drosophila insect with the ability to grow in suspension and serum-free cultures were used for the expression of factor IX (FIX) using Transient Gene Expression (TGE) technique. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6252033PMC
December 2018

Measurement of extended half-life recombinant factor IX products in clinical practice.

Int J Lab Hematol 2018 Dec 7. Epub 2018 Dec 7.

Department Of Coagulation, Royal Hallamshire Hospital, Sheffield, UK.

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http://dx.doi.org/10.1111/ijlh.12953DOI Listing
December 2018
1 Read

Heme oxygenase-1 inducer and carbon monoxide-releasing molecule enhance the effects of gabapentinoids by modulating glial activation during neuropathic pain in mice.

Pain Rep 2018 Sep-Oct;3(5):e677. Epub 2018 Aug 6.

Department of Anesthesiology and Critical Care Medicine, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan.

Introduction: Neuropathic pain is one of the most difficult-to-treat symptoms. Although gabapentinoids are classified as first-line drugs, they have only modest efficacy.

Objectives: The aim of this study was to investigate whether treatment with the heme oxygenase-1 (HO-1) inducer cobalt protoporphyrin IX (CoPP) or the carbon monoxide-releasing molecule tricarbonyldichlororuthenium (II) dimer (CORM-2) can enhance the antinociceptive effects produced by gabapentinoids in mice with neuropathic pain. Read More

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http://dx.doi.org/10.1097/PR9.0000000000000677DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6181470PMC

Potential limits of AAV-based gene therapy with the use of new transgenes expressing factor IX fusion proteins.

Haemophilia 2018 Dec 6. Epub 2018 Dec 6.

Research Department of Haematology, University of London Cancer Institute, London, UK.

Introduction: The variety of treatment for haemophilia B (HB) has recently improved with the emergence of both AAV-based gene therapy and bioengineered human factor IX (hFIX) molecules with prolonged half-life due to fusion to either albumin (Alb) or immunoglobulin Fc fragment (Fc).

Aim: Adeno-associated viral vectors (AAV) mediating expression of hFIX-Alb and hFIX-Fc fusion proteins was investigated for gene therapy of HB to explore if their extended half-life translates to higher plasma levels of FIX.

Methods: Single-stranded cross-packaged AAV2/8 vectors expressing hFIX-Alb, hFIX-Fc and hFIX were evaluated in vitro, and in mice. Read More

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http://dx.doi.org/10.1111/hae.13651DOI Listing
December 2018
2 Reads

Over two decades of orthopaedic surgery in patients with inhibitors-Quantifying the complication of bleeding.

Haemophilia 2018 Dec 3. Epub 2018 Dec 3.

Department of Trauma & Orthopaedics, Royal Free Hospital, London, UK.

Patients with haemophilia who have developed inhibitors against factor VIII (FVIII) or factor IX present a significant concern to those surgeons who operate on them. The evidence base for bypassing agents such as recombinant factor VIIa and activated prothrombin complex concentrate has amassed over several decades. The literature is open to positive interpretation on the successful use of these agents in the treatment of inhibitor-positive patients. Read More

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http://doi.wiley.com/10.1111/hae.13647
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http://dx.doi.org/10.1111/hae.13647DOI Listing
December 2018
8 Reads

Beyond plasma exchange: novel therapies for thrombotic thrombocytopenic purpura.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):539-547

Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.

The advent of plasma exchange has dramatically changed the prognosis of acute thrombotic thrombocytopenic purpura (TTP). Recent insights into TTP pathogenesis have led to the development of novel therapies targeting pathogenic anti-ADAMTS13 antibody production, von Willebrand factor (VWF)-platelet interactions, and ADAMTS13 replacement. Retrospective and prospective studies have established the efficacy of rituximab as an adjunct to plasma exchange for patients with acute TTP, either upfront or for refractory disease. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.539DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246029PMC
November 2018
7 Reads

[Phenotypic and genetic analysis of two pedigrees affected with hereditary coagulation FXII deficiency].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2018 Dec;35(6):800-803

Department of Laboratory Medicine, the Second Affiliated Hospital, Wenzhou Medical University, Wenzhou, Zhejiang 325027, China.

Objective: To carry out phenotypic and genotypic analysis for two Chinese pedigrees affected with coagulation factor XII (F XII) deficiency.

Methods: Plasma prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen (FIB), thrombin time (TT), and blood coagulation factor VIII, IX, XI, XII activity (FVIII:C, FIX:C, FXI:C, FXII:C) were determined with one stage clotting assay on a STAGO coagulation analyzer. FXII antigen was determined with an enzyme linked immunosorbent assay (ELISA). Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2018.06.005DOI Listing
December 2018
1 Read

[Hemin regulates the expression of nuclear factor kappa B of heme oxygenase -1 in hepatic fibrosis].

Zhonghua Gan Zang Bing Za Zhi 2018 Sep;26(9):654-659

Department of Infectious Diseases, First Hospital of Shanxi Medical University, Taiyuan 030001, China.

To observe the therapeutic effects and related mechanism of hemin on the progression of hepatic fibrosis in rats. Sixty male Wistar rats were randomly divided into normal control group, 4-week model group, 6-week model group, hemin inhibitor zinc protoporphyrin-IX (ZnPP-IX) intervention group and hemin intervention group. Hemin intervention group in complex liver fibrosis model was intraperitonealy administered ZnPP-IX or hemin every other day for 2 weeks from the fourth week. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1007-3418.2018.09.004DOI Listing
September 2018
1 Read

Bleeding Disorders in Adolescents with Heavy Menstrual Bleeding: The Queensland Statewide Paediatric and Adolescent Gynaecology Service.

J Pediatr Adolesc Gynecol 2018 Nov 22. Epub 2018 Nov 22.

Statewide Paediatric and Adolescent Gynaecology Service, Royal Brisbane and Women's Hospital and Lady Cilento Children's Hospital, Brisbane, Queensland, Australia; University of Queensland, Faculty of Medicine, Brisbane, Queensland, Australia.

Study Objective: Heavy menstrual bleeding (HMB) is a common gynecological complaint among young women with up to 40% having experienced HMB. Bleeding disorders are increasingly being recognized in adolescents and young adults with HMB. The aim of this study was to determine the prevalence of bleeding disorders in adolescents with HMB, among patients who presented to the Queensland Statewide Paediatric and Adolescent Gynaecology Service between July 2007 and July 2017. Read More

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http://dx.doi.org/10.1016/j.jpag.2018.11.005DOI Listing
November 2018
1 Read

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
9 Reads

The carboxyl-terminal region of human coagulation factor X as a natural linker for fusion strategies.

Thromb Res 2019 Jan 9;173:4-11. Epub 2018 Nov 9.

Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy. Electronic address:

Fusion with human serum albumin (HSA), which represents a well-established technique to extend half-life of therapeutic proteins, commonly exploits intervening peptide linkers as key components. Here, we explored the human coagulation factor X (FX) carboxyl-terminal region, previously demonstrated by us to be dispensable for secretion and coagulant activity, as a natural linker for fusion purposes. To test our hypothesis, we compared direct FX-HSA fusion with the designed FX-HSA fusion proteins mimicking the recombinant activated factor VII (rFVIIa)-HSA or factor IX (FIX)-HSA chimeras, both strongly dependent from artificial linkers. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00493848183060
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http://dx.doi.org/10.1016/j.thromres.2018.11.007DOI Listing
January 2019
6 Reads

Emicizumab, the bispecific antibody to factors IX/IXa and X/Xa, potentiates coagulation function in factor XI-deficient plasma in vitro.

J Thromb Haemost 2019 Jan 10;17(1):126-137. Epub 2018 Dec 10.

Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.

Essentials Emicizumab mimics factor (F)VIIIa cofactor function, augments the intrinsic tenase activity. We assessed the emicizumab-driven hemostatic function in FXI-deficient plasmas. Emicizumab improved the coagulation potentials in severe FXI-deficient plasma. Read More

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http://doi.wiley.com/10.1111/jth.14334
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http://dx.doi.org/10.1111/jth.14334DOI Listing
January 2019
8 Reads

The definition, diagnosis and management of mild hemophilia A: communication from the SSC of the ISTH.

J Thromb Haemost 2018 Dec 15;16(12):2530-2533. Epub 2018 Nov 15.

Department of Pediatric Hematology, Academic Medical Centre, Amsterdam, the Netherlands.

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http://dx.doi.org/10.1111/jth.14315DOI Listing
December 2018
1 Read

Health-related quality of life in paediatric haemophilia B patients treated with rIX-FP.

Haemophilia 2018 Nov 14. Epub 2018 Nov 14.

National Hemophilia Center, Sheba Medical Center, Tel Hashomer, Israel.

Introduction: Frequent infusions and bleeds can impact on the health-related quality of life (HRQoL) of paediatric haemophilia B patients. rIX-FP (IDELVION ) is a fusion protein linking recombinant factor IX with recombinant albumin, and is associated with low bleeding rates with a weekly regimen, which could improve HRQoL.

Aims: To measure the effect of rIX-FP prophylaxis on the HRQoL of paediatric patients and treatment satisfaction in their caregivers using the Haemo-QoL and Hemo-SAT questionnaires, respectively. Read More

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http://doi.wiley.com/10.1111/hae.13624
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http://dx.doi.org/10.1111/hae.13624DOI Listing
November 2018
13 Reads

Carbonic anhydrase inhibitors as emerging agents for the treatment and imaging of hypoxic tumors.

Expert Opin Investig Drugs 2018 Dec 22;27(12):963-970. Epub 2018 Nov 22.

a NEUROFARBA Department, Sezione di Scienze Farmaceutiche e Nutraceutiche , Università degli Studi di Firenze , Sesto Fiorentino (Firenze) , Italy.

Introduction: Hypoxic tumors overexpress two carbonic anhydrases (CA, EC 4.2.1. Read More

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https://www.tandfonline.com/doi/full/10.1080/13543784.2018.1
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http://dx.doi.org/10.1080/13543784.2018.1548608DOI Listing
December 2018
7 Reads

PTH, FGF23, and Intensive Blood Pressure Lowering in Chronic Kidney Disease Participants in SPRINT.

Clin J Am Soc Nephrol 2018 Dec 13;13(12):1816-1824. Epub 2018 Nov 13.

Kidney Health Research Collaborative, Veterans Affairs Medical Center, San Francisco, California; and.

Background And Objectives: The Systolic Blood Pressure Intervention Trial (SPRINT) demonstrated that intensive BP lowering reduced the risk of cardiovascular disease, but increased eGFR decline. Serum parathyroid hormone (PTH) and fibroblast growth factor-23 (FGF23) concentrations are elevated in CKD and are associated with cardiovascular disease. We evaluated whether intact PTH or intact FGF23 concentrations modify the effects of intensive BP control on cardiovascular events, heart failure, and all-cause mortality in SPRINT participants with CKD. Read More

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http://cjasn.asnjournals.org/lookup/doi/10.2215/CJN.05390518
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http://dx.doi.org/10.2215/CJN.05390518DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302330PMC
December 2018
7 Reads

Performance of a recombinant fusion protein linking coagulation factor IX with recombinant albumin in one-stage clotting assays.

J Thromb Haemost 2019 Jan 16;17(1):138-148. Epub 2018 Dec 16.

Blood Research Institute, Blood Center of Wisconsin, Milwaukee, WI, USA.

Essentials Performance of the one-stage clotting (OSC) assay varies with the clotting activator used. Recombinant FIX-albumin fusion protein (rIX-FP) was reliably monitored with most OSC reagents. rIX-FP shows comparable reagent-dependent variability to other rFIX products in the OSC assay. Read More

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http://dx.doi.org/10.1111/jth.14332DOI Listing
January 2019
1 Read

Inhibitors in haemophilia A and B: Management of bleeds, inhibitor eradication and strategies for difficult-to-treat patients.

Eur J Haematol 2019 Feb 6;102(2):111-122. Epub 2018 Dec 6.

Foundation IRCCS Cà Granda, Maggiore Hospital Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy.

The standard therapy for patients with haemophilia is prophylactic treatment with replacement factor VIII (FVIII) or factor IX (FIX). Patients who develop inhibitors against FVIII/FIX face an increased risk of bleeding, and the likelihood of early development of progressive arthropathy, alongside higher treatment-related costs. Bypassing agents can be used to prevent and control bleeding, as well as the recently licensed prophylaxis, emicizumab, but their efficacy is less predictable than that of factor replacement therapy. Read More

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http://doi.wiley.com/10.1111/ejh.13193
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http://dx.doi.org/10.1111/ejh.13193DOI Listing
February 2019
12 Reads

Disease-causing variants of the conserved +2T of 5' splice sites can be rescued by engineered U1snRNAs.

Hum Mutat 2019 Jan 19;40(1):48-52. Epub 2018 Nov 19.

Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.

The ability of variants of the spliceosomal U1snRNA to rescue splicing has been proven in several human disease models, but not for nucleotide changes at the conserved GT nucleotide of 5' splice sites (5'ss), frequent and associated with severe phenotypes. Here, we focused on variants at the 5'ss of F9 intron 3, leading to factor IX (FIX) deficiency (hemophilia B). Through minigene expression, we demonstrated that all changes induce complete exon 3 skipping, which explains the associated hemophilia B phenotype. Read More

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http://dx.doi.org/10.1002/humu.23680DOI Listing
January 2019
6 Reads

Population pharmacokinetics of factor IX in hemophilia B patients undergoing surgery.

J Thromb Haemost 2018 Nov 28;16(11):2196-2207. Epub 2018 Oct 28.

Hospital Pharmacy-Clinical Pharmacology, Academic Medical Center Amsterdam, Amsterdam, the Netherlands.

Essentials Factor IX (FIX) dosing using body weight frequently results in under and overdosing during surgery. We aimed to establish a population pharmacokinetic (PK) model describing the perioperative FIX levels. Population PK parameter values for clearance and V1 were 284 mL h70 kg and 5450 mL70 kg. Read More

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http://doi.wiley.com/10.1111/jth.14292
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http://dx.doi.org/10.1111/jth.14292DOI Listing
November 2018
5 Reads

Supply and demand for hemophilia treatments-Systems-based approaches to mitigate the risk.

Transfus Apher Sci 2018 Dec 30;57(6):731-734. Epub 2018 Oct 30.

Division of Hematology and Oncology, Department of Medicine, St. Michael's Hospital and the University of Toronto, Canada.

Treatment of hemophilia consists of replacement of the missing coagulation factor, either prophylactically or at the time of injury or bleeding. Because of the high cost of these products, which can present a barrier to care, different procurement strategies have been developed at national and regional levels. The emergence of novel therapeutic agents adds complexity to these strategies. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S14730502183043
Publisher Site
http://dx.doi.org/10.1016/j.transci.2018.10.014DOI Listing
December 2018
5 Reads

INCREASED SYSTEMIC HEPARANASE IN RETINAL VEIN OCCLUSION IS ASSOCIATED WITH ACTIVATION OF INFLAMMATION AND THROMBOPHILIA.

Retina 2018 Oct 29. Epub 2018 Oct 29.

Department of Ophthalmology, The First Affiliated Hospital of Kunming Medical University, Kunming, China.

Purpose: To investigate the levels of systemic heparanase, inflammatory markers, and coagulation factor activities in patients with retinal vein occlusion (RVO).

Methods: This prospective study included 18 patients with central RVO, 22 patients with branch RVO, and 40 patients with age-related cataract as the control group. Serum heparanase protein levels and activities were measured by ELISA and a heparan degrading enzyme assay kit, respectively. Read More

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http://dx.doi.org/10.1097/IAE.0000000000002374DOI Listing
October 2018
1 Read

Updates in clinical trial data of extended half-life recombinant factor IX products for the treatment of haemophilia B.

Ther Adv Hematol 2018 Nov 5;9(11):335-346. Epub 2018 Oct 5.

Haemophilia Comprehensive Care Centre, Charlotte Maxeke Johannesburg Academic Hospital, School of Pathology, Faculty of Health Sciences, University of the Witwatersrand and National Health Laboratory Service, 7 York Road, Parktown, Johannesburg 2193, South Africa.

Whilst the global prevalence of haemophilia B is less than that of haemophilia A, rapid and remarkable innovations have been made in the development of haemophilia B therapies in the last decade. The most recent developments are the evolution of extended half-life haemophilia B replacement therapies which are designed to reduce the treatment burden associated with prophylactic infusion of factor IX (FIX) to prevent bleeding in haemophilia B participants. Clinical development programmes have culminated in the completion of three phase III studies on extended half-life (EHL) recombinant FIX (rFIX) products and subsequent approval and registration of these in many countries around the world. Read More

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http://dx.doi.org/10.1177/2040620718802606DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196631PMC
November 2018
5 Reads

Toll-like receptors 2, 4 and 9 and hypoxia markers HIF-1alpha and CAIX in pancreatic intraepithelial neoplasia.

APMIS 2018 Nov;126(11):852-863

Cancer and Translational Medicine Research Unit, Medical Research Center Oulu, University of Oulu and Oulu University Hospital, Oulu, Finland.

Pancreatic cancer arises from precursor lesions called pancreatic intraepithelial neoplasia (PanIN) characterized by inflammatory microenvironment. In pancreatic cancer, strong innate immunity and hypoxia responses are typical. Occurrence and relationship of these responses in human PanINs is unknown. Read More

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http://doi.wiley.com/10.1111/apm.12894
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http://dx.doi.org/10.1111/apm.12894DOI Listing
November 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
1 Read

Unraveling the Role of Heme in Neurodegeneration.

Front Neurosci 2018 9;12:712. Epub 2018 Oct 9.

Molecular Biotechnology Center, Department of Molecular Biotechnology and Health Sciences, University of Torino, Turin, Italy.

Heme (iron-protoporphyrin IX) is an essential co-factor involved in several biological processes, including neuronal survival and differentiation. Nevertheless, an excess of free-heme promotes oxidative stress and lipid peroxidation, thus leading to cell death. The toxic properties of heme in the brain have been extensively studied during intracerebral or subarachnoid hemorrhages. Read More

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https://www.frontiersin.org/article/10.3389/fnins.2018.00712
Publisher Site
http://dx.doi.org/10.3389/fnins.2018.00712DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6189481PMC
October 2018
6 Reads

Coagulation Disturbances in Patients with Argininemia.

Acta Haematol 2018 24;140(4):221-225. Epub 2018 Oct 24.

Division of Nutrition and Metabolism, Department of Pediatrics, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.

Background: Argininemia is an autosomal recessive urea cycle disorder (UCD). Unlike other UCD, hyperammonemia is rarely seen. Patients usually present in childhood with neurological symptoms. Read More

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http://dx.doi.org/10.1159/000493678DOI Listing
October 2018
1 Read

Platelet Signaling in Primary Haemostasis and Arterial Thrombus Formation: Part 1.

Hamostaseologie 2018 Nov 23;38(4):203-210. Epub 2018 Oct 23.

Division of Clinical and Experimental Hemostasis, Hemotherapy and Transfusion Medicine, University Blood Center, and Haemophilia Comprehensive Care Center, Institute of Transplantation Diagnostics and Cell Therapeutics, Heinrich Heine University Medical Center, Düsseldorf, Germany.

Platelets react immediately in response to traumatic vascular injury by adhesion, activation, aggregation and subsequent haemostatic plug formation. While this reaction pattern is essential for haemostasis, platelet responses can also cause occlusive thrombi in diseased arteries, leading to myocardial infarction or stroke. Initially, flowing platelets are captured from the circulation to vascular lesions. Read More

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http://dx.doi.org/10.1055/s-0038-1675144DOI Listing
November 2018
7 Reads

In silico thrombin generation: Plasma composition imbalance and mortality in human immunodeficiency virus.

Res Pract Thromb Haemost 2018 Oct 11;2(4):708-717. Epub 2018 Sep 11.

Department of Medicine University of Minnesota Minneapolis Minnesota USA.

Background: Effective HIV treatment with antiretroviral therapy has prolonged survival and shifted causes of death to non-AIDS illnesses such as cardiovascular disease. We have shown that inflammation and HIV viral load associate with pro- and anticoagulant factor imbalances resulting in increased thrombin generation when mathematically modeled. We explore the hypothesis that factor compositional imbalance, corresponding to increased in silico thrombin generation, predicts mortality among HIV+ persons. Read More

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http://doi.wiley.com/10.1002/rth2.12147
Publisher Site
http://dx.doi.org/10.1002/rth2.12147DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6178732PMC
October 2018
4 Reads

[Clinical and pathological analysis of renal cell carcinoma bone metastasis].

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Oct;50(5):811-815

Department of Urology, Peking University People's Hospital, Beijing 100044, China.

Objective: To analyze the clinical and pathological characteristics of renal cell carcinoma bone metastasis (RCC-BM) patients.

Methods: Data of RCC-BM patients from July 2003 to November 2017 were retrospectively reviewed. The patients' baseline characteristics (age, gender), tumor characteristics [specific sites of bone metastasis, time to bone metastasis (TTBM), imaging features of bone disease, coexistence of other metastasis], as well as pathological features (histological classification of primary and bone metastasis, immunohistochemical stain results) were collected. Read More

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October 2018
1 Read

Clinical utility of viscoelastic testing (TEG and ROTEM analyzers) in the management of old and new therapies for hemophilia.

Am J Hematol 2019 Feb 7;94(2):249-256. Epub 2018 Dec 7.

Wayne State University School of Medicine, Children's Hospital of Michigan, Detroit, Michigan.

Hemophilia A and B are rare inherited bleeding disorders resulting from deficiency of coagulation factors VIII and IX respectively. In the past few decades, the field of hemophilia has witnessed pivotal management challenges and therapeutic advances. Routine coagulation and factor assays, while useful in the classification of severity and treatment monitoring in hemophilia patients, have been shown to be of limited use in managing clinical presentations and outcomes. Read More

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http://dx.doi.org/10.1002/ajh.25319DOI Listing
February 2019
1 Read

Rosmarinic acid elicits neuroprotection in ischemic stroke Nrf2 and heme oxygenase 1 signaling.

Neural Regen Res 2018 Dec;13(12):2119-2128

Department of Neurology, Second Hospital of Hebei Medical University; Hebei Key Laboratory of Vascular Homeostasis and Hebei Collaborative Innovation Center for Cardiocerebrovascular Disease, Shijiazhuang, Hebei Province, China.

Rosmarinic acid (RA) can elicit a neuroprotective effect against ischemic stroke, but the precise molecular mechanism remains poorly understood. In this study, an experimental ischemic stroke model was established in CD-1 mice (Beijing Vital River Laboratory Animal Technology, Beijing, China) by occluding the right middle cerebral artery for 1 hour and allowing reperfusion for 24 hours. After intraperitoneally injecting model mice with 10, 20, or 40 mg/kg RA, functional neurological deficits were evaluated using modified Longa scores. Read More

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http://www.nrronline.org/text.asp?2018/13/12/2119/241463
Publisher Site
http://dx.doi.org/10.4103/1673-5374.241463DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6199925PMC
December 2018
8 Reads

[Development of gene therapy for hemophilia: current states and future perspectives].

Authors:
Tsukasa Ohmori

Rinsho Ketsueki 2018;59(10):2238-2246

Department of Biochemistry, Jichi Medical University.

Hemophilia is congenital hemorrhagic disease due to genetic abnormality of blood coagulation factor VIII or factor IX. Hemophilia appears suitable for gene therapy because it is caused by a single gene abnormality, and therapeutic coagulation factor levels vary across a broad range. Since the success of gene therapy eliminates the need for regular administration of factor concentrates, the development has been met with great expectation from patients and their families. Read More

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https://www.jstage.jst.go.jp/article/rinketsu/59/10/59_2238/
Publisher Site
http://dx.doi.org/10.11406/rinketsu.59.2238DOI Listing
January 2018
3 Reads