نتایج جستجو برای: concentrated fviii

تعداد نتایج: 43809  

Journal: :The Journal of clinical investigation 2015
Davide Matino Marco Gargaro Elena Santagostino Matteo N D Di Minno Giancarlo Castaman Massimo Morfini Angiola Rocino Maria E Mancuso Giovanni Di Minno Antonio Coppola Vincenzo N Talesa Claudia Volpi Carmine Vacca Ciriana Orabona Rossana Iannitti Maria G Mazzucconi Cristina Santoro Antonella Tosti Sara Chiappalupi Guglielmo Sorci Giuseppe Tagariello Donata Belvini Paolo Radossi Raffaele Landolfi Dietmar Fuchs Louis Boon Matteo Pirro Emanuela Marchesini Ursula Grohmann Paolo Puccetti Alfonso Iorio Francesca Fallarino

The development of inhibitory antibodies to factor VIII (FVIII) is a major obstacle in using this clotting factor to treat individuals with hemophilia A. Patients with a congenital absence of FVIII do not develop central tolerance to FVIII, and therefore, any control of their FVIII-reactive lymphocytes relies upon peripheral tolerance mechanisms. Indoleamine 2,3-dioxygenase 1 (IDO1) is a key re...

Journal: :Haemophilia : the official journal of the World Federation of Hemophilia 2009
S W Pipe

Unravelling the structure, function and molecular interactions of factor VIII (FVIII) throughout its life cycle from biosynthesis to clearance has advanced our understanding of the molecular mechanisms of haemophilia and the development of effective treatment strategies including recombinant replacement therapy. These insights are now influencing bioengineering strategies toward novel therapeut...

Journal: :Blood 1979
N W Stead P A McKee

Cultured human umbilical vein endothelial cells produce a protein that has von Willebrand factor activity and forms immunoprecipitates with rabbit antibody to purified plasma factor VIII/von Willebrand factor (FVIII/vWF) protein, but it has no FVIII procoagulant activity. Of the three characteristics of plasma FVIII/vWF protein, only FVIII procoagulant activity is readily destroyed by trace pro...

2017
A. Pavlova

Correspondence to: Dr. Anna Pavlova Institut für Experimentelle Hämatologie und Transfusionsmedizin , Universitätsklinikum Bonn Sigmund-Freud-Str. 25, 53127 Bonn, Germany Tel. +49/(0)/228/28 71 97 11, Fax +49/(0)228/28 71 60 87 E-mail: [email protected] Hämostaseologie 2010; 30 (Suppl 1): S153–S155 Human blood coagulation factor VIII (FVIII) is an essential protein for the blood coag...

2007
Christopher Parmenter Svetla Stoilova-McPhie

Membrane-binding of a human blood clotting protein, factor VIII (FVIII), was studied by cryoelectron microscopy. Well visible gap junctions between adjacent phosphatydyl serine containing phospholipid vesicles were observed upon FVIII association. Digital images acquired with a JEOL2010FEG transmission electron microscope equipped with a 4x4k CCD camera were analysed with the Digital Micrograph...

Journal: :Vox sanguinis 2002
E L Saenko N Ananyeva D Kouiavskaia H Schwinn D Josic M Shima C A E Hauser S Pipe

Molecular defects in Factor VIII (FVIII), such as haemophilia A-related mutations or denaturative conformational changes, may affect the stability of FVIII as well as its interactions with physiological activators, von Willebrand Factor, phospholipid, or conformationally sensitive antibodies. We summarize the contemporary assays which allow identification of impaired functional interactions of ...

2003
Hui-Chi Hsu Chao-Hung Ho Wing-Keung Chau Chih-Cheng Chen Jie-Yu You

dis or der af fect ing ap prox i mately 1 in 10,000 male. The dis ease is caused by a de fi ciency of co ag u la tion fac tor VIII (FVIII) re sult ing from mu ta tions in the FVIII gene. Nu cle o tide sub sti tu tions/in ser tions of var i ous sizes have been iden ti fied through out the FVIII gene. This ge netic di ver sity ac counts for the ob served het er o ge ne ity in clin i cal se ver it...

2017
Noémi Klára Tóth Edina Gabriella Székely Katalin Réka Czuriga-Kovács Ferenc Sarkady Orsolya Nagy Levente István Lánczi Ervin Berényi Klára Fekete István Fekete László Csiba Zsuzsa Bagoly

Introduction Plasma factor VIII (FVIII) and von Willebrand factor (VWF) levels have been associated with the rate and severity of arterial thrombus formation and have been linked to outcomes following thrombolytic therapy in acute myocardial infarction patients. Here, we aimed to investigate FVIII and VWF levels during the course of thrombolysis in acute ischemic stroke (AIS) patients and to fi...

2014
Lesley A. Everett Audrey C.A. Cleuren Rami N. Khoriaty David Ginsburg

The primary cellular source of FVIII biosynthesis is controversial, with contradictory evidence supporting an endothelial or hepatocyte origin. LMAN1 is a cargo receptor in the early secretory pathway that is responsible for the efficient secretion of FV and FVIII to the plasma. Lman1 mutations result in combined deficiency of FV and FVIII, with levels of both factors reduced to ~10-15% of norm...

Journal: :Haemophilia : the official journal of the World Federation of Hemophilia 2010
J S Stonebraker M Brooker R E Amand A Farrugia A Srivastava

The effect of replacement therapy has significantly improved the morbidity and mortality of people with haemophilia A in high income countries, a recent socio-economic development as the availability of safe concentrates has been matched by a willingness for their provision through reimbursement. In the developing world, however, this state has not been achieved, primarily because of the low vi...

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