Results 11 to 20 of about 16,303 (230)

Co-administration of FVIII with IVIG reduces immune response to FVIII in hemophilia A mice [PDF]

Scientific Reports, 2022
AbstractHemophilia A is an X-linked recessive congenital bleeding disorder. Exogenous infusion of FVIII is the treatment of choice, and the development of immunoglobulins against FVIII (inhibitors) remains the major challenge in clinical management of the disease.
Sajjad Afraz, Ivan Stevic, Davide Matino, Jianping Wen, Helen Atkinson, Anthony K. C. Chan, Gonzalo Hortelano   +6 more
openaire   +4 more sources

Some Factor VIII (FVIII) Inhibitors Recognise a FVIII Epitope(s) that Is Present only on FVIII-vWF Complexes

Thrombosis and Haemostasis, 1999
SummaryA mild haemophilia A patient (LE) with an Arg2150His mutation in the C1 domain of the factor VIII (FVIII) light chain was shown to have anti-FVIII antibodies inhibiting wild type but not self FVIII. Polyclonal anti-FVIII antibodies of this patient were purified by affinity adsorption using recombinant FVIII (rFVIII) and/or plasma-derived FVIII ...
J G, Gilles, R, Lavend'homme, K, Peerlinck, M G, Jacquemin, M, Hoylaerts, S, Jorieux, C, Mazurier, J, Vermylen, J M, Saint-Remy   +8 more
openaire   +3 more sources

In-Silico Characterization of von Willebrand Factor Bound to FVIII

Applied Sciences, 2022
Factor VIII belongs to the coagulation cascade and is expressed as a long pre-protein (mature form, 2351 amino acids long). FVIII is deficient or defective in hemophilic A patients, who need to be treated with hemoderivatives or recombinant FVIII ...
Valentina Drago, Luisa Di Paola, Claire Lesieur, Renato Bernardini, Claudio Bucolo, Chiara Bianca Maria Platania   +5 more
doaj   +2 more sources

FVIII Immunity : early events and tolerance mechanisms to FVIII [PDF]

, 2008
Among the complications of current treatments for hemophilia A, the development of anti-FVIII antibodies including “FVIII inhibitors” remains the major clinical problem in treating hemophiliacs. Factor VIII inhibitors work through neutralizing the coagulation cofactor activity of the infused FVIII and preventing the restoration of normal hemostasis ...
Qadura, Mohammad Imad
openaire   +2 more sources

Combined FV and FVIII deficiency

Haemophilia, 2008
Summary.  Inherited deficiencies of plasma proteins involved in blood coagulation generally lead to lifelong bleeding disorders. The severity of these disorders is generally inversely proportional to the degree of factor deficiency. Among all the autosomal recessive rare bleeding disorders, which include afibrinogenaemia, factor (F) II, FV, FV + VIII ...
M. Spreafico, F. Peyvandi
openaire   +4 more sources

Current Understanding of Inherited Modifiers of FVIII Pharmacokinetic Variation

Pharmacogenomics and Personalized Medicine, 2023
Laura L Swystun, David Lillicrap Department of Pathology and Molecular Medicine, Queen’s University, Kingston, ON, CanadaCorrespondence: David Lillicrap, Richardson Laboratory, Queen’s University, 88 Stuart Street, Kingston, Ontario, K7L 3N6, Canada, Tel
Swystun LL, Lillicrap D
doaj   +1 more source

Chemical chaperones improve protein secretion and rescue mutant factor VIII in mice with hemophilia A. [PDF]

PLoS ONE, 2012
Inefficient intracellular protein trafficking is a critical issue in the pathogenesis of a variety of diseases and in recombinant protein production.
Stefanie D Roth, Jörg Schüttrumpf, Peter Milanov, Daniela Abriss, Christopher Ungerer, Patricia Quade-Lyssy, Jeremy C Simpson, Rainer Pepperkok, Erhard Seifried, Torsten Tonn   +9 more
doaj   +2 more sources

FVIII hydrolysis mediated by anti-FVIII autoantibodies in acquired hemophilia

, 2008
Acquired hemophilia is a rare hemorrhagic disorder caused by the spontaneous appearance of inhibitory autoantibodies directed against endogenous coagulation factor VIII (FVIII). Inhibitory Abs also arise in patients with congenital hemophilia A as alloantibodies directed to therapeutic FVIII.
Wootla, Bharath, Dasgupta, Suryasarathi, Dimitrov, Jordan, Bayry, Jagadeesh, Lévesque, Hervé, Borg, Jeanne-Yvonne, Borel-Derlon, Annie, Rao, Desirazu, Friboulet, Alain, Kaveri, Srinivas, Lacroix-Desmazes, Sébastien   +10 more
openaire   +3 more sources

Synovial Gene expression after Hemarthrosis differs between FVIII-deficient mice treated with recombinant FVIII or FVIII-Fc Fusion Protein

PLOS One
To investigate if FVIII-Fc Fusion protein (FcFVIII) may modulate inflammation and immune stimulation in hemophilic synovium via the Fc-portion of immunoglobulin used for half-life extension we performed gene expression profiling in FVIII-deficient mice.
Bilgimol Chumappumkal Joseph, Thomas C. Whisenant, Esther J. Cooke, Jenny Y. Zhou, Nicca Falah, Juan Andres De-Pablo Moreno, Annette von Drygalski   +6 more
openaire   +5 more sources

Patient anti-FVIII drug antibodies bind preferentially to a subset of FVIII covalent states

Blood Advances
: Hemophilia A is a chronic life-threatening condition caused by the deficiency or dysfunction of plasma coagulation factor VIII (FVIII) and commonly managed by prophylaxis with regular infusion of FVIII protein.
Diego Butera, Aster E. Pijning, Nathan G. Avery, Carmen H. Coxon, Clive Metcalfe, Angelina Mimoun, Sébastien Lacroix-Desmazes, Paul Clint Spiegel, Jr., Philip J. Hogg   +8 more
doaj   +3 more sources