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1. Full Text AAV5–Factor VIII Gene Transfer in Severe Hemophilia A
by Rangarajan, Savita and Walsh, Liron and Lester, Will and Perry, David and Madan, Bella and Laffan, Michael and Yu, Hua and Vettermann, Christian and Pierce, Glenn F and Wong, Wing Y and Pasi, K. John
The New England Journal of Medicine, ISSN 0028-4793, 12/2017, Volume 377, Issue 26, pp. 2519 - 2530
An AAV5 vector with a functional factor VIII gene was tested in men with hemophilia A. Men receiving the high dose had a factor VIII level above 5 IU per... 
TRIAL | PROPHYLAXIS | RATES | MEDICINE, GENERAL & INTERNAL | THERAPY | MANAGEMENT | FACTOR-IX | MICE | VARIANT | Hemophilia A - immunology | Humans | Factor VIII - genetics | Dependovirus - immunology | Male | Hemorrhage - prevention & control | Virus Shedding | Hemophilia A - therapy | Antibodies, Viral - blood | Young Adult | Hemophilia A - metabolism | Factor VIII - administration & dosage | Adult | Hemophilia A - genetics | Factor VIII - metabolism | Infusions, Intravenous | Genetic Vectors | DNA, Viral | Genetic Therapy - adverse effects | Genetic Therapy - methods | Treatment outcome | Care and treatment | Genetic aspects | Analysis | Hemophilia | Coagulation factors | Alanine | Intravenous administration | Hemostasis | Alanine transaminase | Genomes | Gene therapy | Bleeding | Quality of life | Pharmaceuticals | Index Medicus | Abridged Index Medicus
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2. Full Text Selective factor VIII activation by the tissue factor–factor VIIa–factor Xa complex
by Kamikubo, Yuichi and Mendolicchio, G. Loredana and Zampolli, Antonella and Marchese, Patrizia and Rothmeier, Andrea S and Orje, Jennifer Nagrampa and Gale, Andrew J and Krishnaswamy, Sriram and Gruber, András and Østergaard, Henrik and Petersen, Lars C and Ruf, Wolfram and Ruggeri, Zaverio M
Blood, ISSN 0006-4971, 10/2017, Volume 130, Issue 14, pp. 1661 - 1670
Safe and effective antithrombotic therapy requires understanding of mechanisms that contribute to pathological thrombosis but have a lesser impact on... 
THROMBIN GENERATION | COAGULATION-FACTOR VIIA | FACTOR-XI ACTIVATION | INITIATION | FACTOR-IX | FACTOR-V | EXTRINSIC PATHWAY | ARTERIAL THROMBOSIS | PROTEIN-C | HEMATOLOGY | FACTOR PATHWAY INHIBITOR | Factor VIIIa - metabolism | Humans | Blood Coagulation | Factor VIII - metabolism | Thrombin - metabolism | Thromboplastin - metabolism | Factor VIIa - metabolism | Factor Xa - metabolism | Index Medicus | Abridged Index Medicus | 37 | Thrombosis and Hemostasis
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3. Full Text Human liver sinusoidal endothelial cells but not hepatocytes contain factor VIII
by Shahani, T and Covens, K and Lavend'homme, R and Jazouli, N and Sokal, E and Peerlinck, K and Jacquemin, M
Journal of Thrombosis and Haemostasis, ISSN 1538-7933, 01/2014, Volume 12, Issue 1, pp. 36 - 42
BackgroundAlthough the liver is the major site of coagulation factorVIII (FVIII) synthesis, the type of cells producing FVIII within the liver is still... 
coagulation factor | hemophilia | endothelial cells | VIII | liver | hepatocytes | Coagulation factor VIII | Hepatocytes | Hemophilia A | Liver | Endothelial cells | LOCALIZATION | coagulation factorVIII | TISSUE | PROCOAGULANT ANTIGEN | PHENOTYPE | TRANSPLANTATION | HEMOPHILIA-A MICE | PERIPHERAL VASCULAR DISEASE | hemophiliaA | SECRETION | MONOCLONAL-ANTIBODIES | HEMATOLOGY | EXPRESSION | Sequence Analysis, RNA | Endothelial Cells - metabolism | Humans | Liver - metabolism | Liver - cytology | Factor VIII - metabolism | Hepatocytes - metabolism | Blood coagulation factor VIII | RNA | Hemophilia | Endothelium | Index Medicus
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4. Full Text Factor VIII and von Willebrand factor are ligands for the carbohydrate-receptor Siglec-5
by Julie N. Pegon and Mohamad Kurdi and Caterina Casari and Soline Odouard and Cécile V. Denis and Olivier D. Christophe and Peter J. Lenting
Haematologica, ISSN 0390-6078, 12/2012, Volume 97, Issue 12, pp. 1855 - 1863
Background Factor VIII (FVIII) and von Willebrand factor (VWF) circulate in plasma in a tight non-covalent complex, being critical to hemostasis. Although... 
Ligand | Siglec-5 | Interaction | von Willebrand factor | Factor VIII | CELLS | PROTEIN | RECOGNITION | COAGULATION-FACTOR-VIII | ligand | PLASMA | interaction | PHAGOCYTES | CHAINS | GROUP-B | HEMATOLOGY | factor VIII | CD33-RELATED SIGLECS | EXPRESSION | Monocytes - cytology | Humans | Mice, Inbred C57BL | Cells, Cultured | Monocytes - metabolism | Kidney - cytology | Hepatocytes - metabolism | Macrophages - cytology | Endosomes - metabolism | Immunoenzyme Techniques | Antigens, CD - metabolism | Polysaccharides - metabolism | Lectins - metabolism | Endocytosis | Hepatocytes - cytology | Kidney - metabolism | Macrophages - metabolism | Animals | Antigens, Differentiation, Myelomonocytic - metabolism | Mice | Factor VIII - metabolism | von Willebrand Factor - metabolism | Index Medicus | Original and Brief Reports
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5. Full Text Definitions in hemophilia: Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the international society on thrombosis and haemostasis
by White II, G.C and Rosendaal, F and Aledort, L.M and Lusher, J.M and Rothschild, C and Ingerslev, J and Factor VIII Factor IX Subcommittee and Factor VIII and Factor IX Subcommittee
Thrombosis and Haemostasis, ISSN 0340-6245, 2001, Volume 85, Issue 3, pp. 560 - 560
HEMATOLOGY | PERIPHERAL VASCULAR DISEASE | Severity of Illness Index | Factor VIII - metabolism | Hemophilia A - classification | Hemophilia A - diagnosis | Humans | Index Medicus
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6. Full Text Oxidized Phospholipids Induce Type VIII Collagen Expression and Vascular Smooth Muscle Cell Migration
by Cherepanova, Olga A and Pidkovka, Nataliya A and Sarmento, Olga F and Yoshida, Tadashi and Gan, Qiong and Adiguzel, Eser and Bendeck, Michelle P and Berliner, Judith and Leitinger, Norbert and Owens, Gary K
Circulation Research, ISSN 0009-7330, 03/2009, Volume 104, Issue 5, pp. 609 - 618
Phenotypic switching of vascular smooth muscle cells (VSMCs) is known to play a critical role in the development of atherosclerosis. However, the factors... 
PEIPC | Type VIII collagen | Klf4 | POVPC | PGPC | Vascular smooth muscle cell migration | TRANSCRIPTION FACTORS | CARDIAC & CARDIOVASCULAR SYSTEMS | DIFFERENTIAL EXPRESSION | LOW-DENSITY-LIPOPROTEIN | vascular smooth muscle cell migration | CAROTID ARTERIES | type VIII collagen | IN-VIVO | GROWTH | GENE-EXPRESSION | PERIPHERAL VASCULAR DISEASE | EXTRACELLULAR-MATRIX | KRUPPEL-LIKE FACTOR | HEMATOLOGY | MARKER GENES | Carotid Arteries - metabolism | Up-Regulation | Muscle, Smooth, Vascular - metabolism | Male | Sp1 Transcription Factor - metabolism | Aorta - metabolism | Phosphatidylcholines - metabolism | RNA, Messenger - metabolism | Apolipoproteins E - metabolism | Transfection | RNA Interference | Time Factors | Kruppel-Like Transcription Factors - metabolism | Lipoproteins, LDL - metabolism | Phospholipids - metabolism | Myocytes, Smooth Muscle - metabolism | Promoter Regions, Genetic | Phospholipid Ethers - metabolism | Oxidation-Reduction | Mice, Inbred C57BL | Cells, Cultured | Rats | Mice, Knockout | Phenotype | Animals | Apolipoproteins E - genetics | Collagen Type VIII - metabolism | Mice | Kruppel-Like Transcription Factors - genetics | Cell Movement | Collagen Type VIII - genetics | RNA, Small Interfering - metabolism | Index Medicus
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7. Full Text Production of recombinant factor VIII in human cell lines
by Fantacini, Daianne Maciely Carvalho and Picanço-Castro, Virgínia
Methods in Molecular Biology, ISSN 1064-3745, 2018, Volume 1674, pp. 63 - 74
Human cell lines can produce recombinant proteins much more similar to their natural counterpart, compared to other mammalian cell lines, reducing potential... 
Human cell line | Lentiviral vector | Transduction | Recombinant FVIII | SK-HEP | Lentivirus - metabolism | Recombinant Proteins - metabolism | Cell Line | Animals | Epitopes - metabolism | Neuraminic Acids - metabolism | Humans | Galectin 3 - metabolism | HEK293 Cells | Antigens - metabolism | Glycosylation | Factor VIII - metabolism | Index Medicus
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8. Full Text Structural characterization of carbohydrate binding by LMAN1 protein provides new insight into the endoplasmic reticulum export of factors V (FV) and VIII (FVIII)
by Zheng, Chunlei and Page, Richard C and Das, Vaijayanti and Nix, Jay C and Wigren, Edvard and Misra, Saurav and Zhang, Bin
Journal of Biological Chemistry, ISSN 0021-9258, 07/2013, Volume 288, Issue 28, pp. 20499 - 20509
LMAN1 (ERGIC-53) is a key mammalian cargo receptor responsible for the export of a subset of glycoproteins from the endoplasmic reticulum. Together with its... 
HELA-CELLS | GOLGI INTERMEDIATE COMPARTMENT | TRANSPORT RECEPTOR COMPLEX | CRYSTAL-STRUCTURE | COMBINED DEFICIENCY | BIOCHEMISTRY & MOLECULAR BIOLOGY | LECTIN ERGIC-53 | COAGULATION-FACTOR VIII | RECOGNITION DOMAIN | MOLECULAR-BASIS | SECRETORY PATHWAY | Mannose-Binding Lectins - metabolism | Glycine - metabolism | Calcium - metabolism | Vesicular Transport Proteins - metabolism | Humans | Cercopithecus aethiops | Endoplasmic Reticulum - metabolism | Molecular Sequence Data | Crystallography, X-Ray | Histidine - metabolism | Mannose - metabolism | Membrane Proteins - metabolism | Mannose - chemistry | Carbohydrate Sequence | Glycine - genetics | Protein Structure, Tertiary | Factor V - metabolism | Membrane Proteins - genetics | Vesicular Transport Proteins - genetics | Factor VIII - genetics | Histidine - genetics | Models, Molecular | Binding Sites - genetics | Blotting, Western | Mannose-Binding Lectins - chemistry | Animals | Mannose-Binding Lectins - genetics | Membrane Proteins - chemistry | Protein Binding | Golgi Apparatus - metabolism | Factor V - genetics | Factor VIII - metabolism | Mutation | COS Cells | Index Medicus | Membrane Trafficking | Protein Secretion | Carbohydrate-binding Protein | Hemostasis | ERGIC | Hemorrhagic Disorders | Calcium-binding Proteins | Coagulation Factors | Golgi | Cell Biology | Endoplasmic Reticulum (ER)
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9. Full Text One-stage factor VIII assays
by Duncan, Elizabeth and Rodgers, Susan
Methods in Molecular Biology, ISSN 1064-3745, 2017, Volume 1646, pp. 247 - 263
Coagulation factor assays using one-stage methodology are widely used to measure factor levels for the purpose of detecting a deficiency, or to monitor... 
Factor XI | Factor IX | Coagulation | Factor VIII | Hemophilia | Factor VIII - analysis | Factor IX - analysis | Factor XII - analysis | Humans | Hemophilia A - blood | Hemophilia A - metabolism | Partial Thromboplastin Time - methods | Factor XII - metabolism | Blood Coagulation | Factor IX - metabolism | Factor VIII - metabolism | Hemophilia A - diagnosis | Quality Control | Index Medicus
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10. Full Text Marginal zone B cells are critical to factor VIII inhibitor formation in mice with hemophilia A
by Zerra, Patricia E and Cox, Courtney and Baldwin, W. Hunter and Patel, Seema R and Arthur, Connie M and Lollar, Pete and Meeks, Shannon L and Stowell, Sean R
Blood, ISSN 0006-4971, 12/2017, Volume 130, Issue 23, pp. 2559 - 2568
Although factor VIII (FVIII) replacement therapy can be lifesaving for patients with hemophilia A, neutralizing alloantibodies to FVIII, known as inhibitors,... 
Factor VIII - immunology | Spleen - immunology | Hemophilia A - immunology | Factor VIII - genetics | Male | Lymphocyte Depletion | Hemophilia A - blood | Mice, Knockout | Protein Transport | Isoantibodies - blood | Animals | B-Lymphocytes - immunology | Spleen - metabolism | Isoantibodies - immunology | Female | Hemophilia A - genetics | Mice | Factor VIII - metabolism | B-Lymphocytes - metabolism | Disease Models, Animal | Index Medicus | Abridged Index Medicus | Thrombosis and Hemostasis
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11. Full Text Safety of PEGylated recombinant human full‐length coagulation factor VIII (BAX 855) in the overall context of PEG and PEG conjugates
by Stidl, R and Fuchs, S and Bossard, M and Siekmann, J and Turecek, P. L and Putz, M
Haemophilia, ISSN 1351-8216, 01/2016, Volume 22, Issue 1, pp. 54 - 64
Introduction: BAX 855 is a PEGylated human full-length recombinant factor VIII (rFVIII) based on licensed rFVIII (ADVATE). The applied PEGylation technology... 
PEG | safety | 855 | haemophilia | BAX | VIII | factor | conjugation | Haemophilia | Conjugation | Safety | BAX 855 | Factor VIII | EFFICACY | VACUOLATION | OPPORTUNITIES | EXCRETION | POLYETHYLENE-GLYCOLS | CHALLENGES | PROFILE | METABOLISM | LONGER-ACTING RFVIII | PROTEINS | HEMATOLOGY | factor VIII | Rabbits | Factor VIII - pharmacokinetics | Humans | Factor VIII - chemistry | Polyethylene Glycols - adverse effects | Rats | Male | Polyethylene Glycols - chemistry | Vacuoles - drug effects | Tissue Distribution | Animals | Vacuoles - metabolism | Factor VIII - adverse effects | Female | Factor VIII - metabolism | Biological products | Safety and security measures | Blood coagulation factor VIII | Polyols | Hemophilia | Index Medicus | Original
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12. Full Text Uptake of blood coagulation factor VIII by dendritic cells is mediated via its C1 domain
by Herczenik, Eszter, PhD and van Haren, Simon D., MSc and Wroblewska, Aleksandra, MSc and Kaijen, Paul, MSc and van den Biggelaar, Maartje, PhD and Meijer, Alexander B., PhD and Martinez-Pomares, Luisa, PhD and ten Brinke, Anja, PhD and Voorberg, Jan, PhD
Journal of Allergy and Clinical Immunology, The, ISSN 0091-6749, 2011, Volume 129, Issue 2, pp. 501 - 509.e5
Background The uptake and processing of blood coagulation factor VIII (FVIII) by antigen-presenting cells and the subsequent presentation of FVIII-derived... 
Allergy and Immunology | coagulation factor VIII | endocytosis | antibody formation | dendritic cells | Hemophilia | IMMUNE-RESPONSE | RECEPTOR-RELATED PROTEIN | IMMUNOLOGY | VON-WILLEBRAND-FACTOR | ENDOGENOUS LIGANDS | FACTOR IXA | MURINE HEMOPHILIA | HUMAN-ANTIBODIES | MANNOSE RECEPTOR | ALLERGY | INHIBITOR DEVELOPMENT | HEMOPHILIA-A PATIENT | Mannose-Binding Lectins - metabolism | Protein Structure, Tertiary | Antibodies, Monoclonal - pharmacology | Humans | Low Density Lipoprotein Receptor-Related Protein-1 - metabolism | Receptors, Cell Surface - metabolism | Cell Adhesion Molecules - metabolism | Mice, Knockout | Hemophilia A - metabolism | Lectins, C-Type - metabolism | Animals | Mice | Factor VIII - metabolism | Binding Sites | Dendritic Cells - metabolism | Blood coagulation factor VIII | Dendritic cells | Peptides | Low density lipoproteins | T cells | Sugars | Monosaccharides | Proteins | Experiments | Blood | Binding sites | Index Medicus | Abridged Index Medicus
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13. Full Text Factor VIII/V C-domain swaps reveal discrete C-domain roles in factor VIII function and intracellular trafficking
by Ebberink, Eduard H. T. M and Bouwens, Eveline A. M and Bloem, Esther and Boon-Spijker, Mariëtte and van den Biggelaar, Maartje and Voorberg, Jan and Meijer, Alexander B and Mertens, Koen
Haematologica, ISSN 0390-6078, 2017, Volume 102, Issue 4, pp. 686 - 694
Factor VIII C-domains are believed to have specific functions in cofactor activity and in interactions with von Willebrand factor. We have previously shown... 
FACTOR IXA | LYSINE RESIDUES | WEIBEL-PALADE BODIES | FACTOR-VA | COFACTOR FUNCTION | CRYSTAL-STRUCTURE | COAGULATION-FACTOR-VIII | ENDOTHELIAL-CELLS | HEMATOLOGY | VON-WILLEBRAND-FACTOR | BINDING | Gene Expression | Factor V - metabolism | Endothelial Cells - metabolism | Humans | Factor VIII - chemistry | Factor VIII - genetics | Models, Molecular | Factor V - chemistry | Structure-Activity Relationship | Protein Transport | Intracellular Space - metabolism | Protein Binding | Protein Conformation | Factor V - genetics | Protein Interaction Domains and Motifs | Factor VIII - metabolism | von Willebrand Factor - metabolism | Index Medicus
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14. Full Text Galectin-1 and Galectin-3 Constitute Novel-Binding Partners for Factor VIII
by O’Sullivan, Jamie M and Jenkins, P Vince and Rawley, Orla and Gegenbauer, Kristina and Chion, Alain and Lavin, Michelle and Byrne, Barry and O’Kennedy, Richard and Preston, Roger J.S and Brophy, Teresa M and O’Donnell, James S
Arteriosclerosis, Thrombosis, and Vascular Biology, ISSN 1079-5642, 05/2016, Volume 36, Issue 5, pp. 855 - 863
OBJECTIVE—Recent studies have demonstrated that galectin-1 (Gal-1) and galectin-3 (Gal-3) can bind von Willebrand factor and directly modulate von Willebrand... 
glycosylation | galectins | plasma | factor VIII | lectins | Recombinant Proteins - metabolism | Cricetulus | Humans | Factor VIII - chemistry | Galectin 3 - metabolism | Factor VIII - genetics | Galectin 1 - metabolism | Glycosylation | Animals | Transfection | Partial Thromboplastin Time | Blood Coagulation | Protein Binding | Protein Conformation | Galectin 1 - chemistry | Galectin 3 - chemistry | Factor VIII - metabolism | Binding Sites | CHO Cells | Index Medicus
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