Abstract
The studies we reviewed here have begun to clarify the complex cellular mechanisms involved in the immune response to fVIII, and the circumstances under which fVIII inhibitors develop. Further characterization and comparison of the immune response to fVIII in both hemophilia patients and healthy subjects will help to further elucidate these mechanisms. The murine hemophilia model will hopefully provide further insights into the mechanisms of inhibitor formation, and prove to be a suitable tool for the design and testing of therapeutic strategies aimed at preventing the development of fVIII inhibitors.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
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Review
MeSH terms
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Animals
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Antibody Specificity
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Autoantibodies / immunology
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CD4-Positive T-Lymphocytes / immunology*
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Desensitization, Immunologic
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Disease Models, Animal
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Epitopes / chemistry
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Epitopes / immunology
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Factor VIII / chemistry
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Factor VIII / immunology*
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Factor VIII / therapeutic use
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Feasibility Studies
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Hemophilia A / immunology*
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Hemophilia A / therapy
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Humans
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Immune Tolerance
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Immunosuppression Therapy / methods
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Interleukin-4 / deficiency
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Interleukin-4 / genetics
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Interleukin-4 / physiology
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Isoantibodies / biosynthesis*
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Isoantibodies / immunology
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Lymphocyte Cooperation*
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Male
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Mice
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Mice, Inbred C57BL
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Mice, Knockout
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Myasthenia Gravis / immunology
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Myasthenia Gravis / therapy
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Peptide Fragments / immunology
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Protein Structure, Tertiary
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Receptors, Cholinergic / immunology
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Th1 Cells / immunology
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Th2 Cells / immunology
Substances
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Autoantibodies
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Epitopes
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Isoantibodies
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Peptide Fragments
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Receptors, Cholinergic
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Interleukin-4
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Factor VIII