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Article
Open AccessA subset of patients with systemic lupus erythematosus fails to degrade DNA from multiple clinically relevant sources
Patients with systemic lupus erythematosus (SLE) have a decreased ability to clear cell remnants and multiple deficiencies in the ability to degrade cellular chromatin have been linked to the disease. Since th...
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Article
Local expression of complement factor I in breast cancer cells correlates with poor survival and recurrence
Tumor cells often evade killing by the complement system by overexpressing membrane-bound complement inhibitors. However, production of soluble complement inhibitors in cells other than hepatocytes was rarely ...
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Protocol
Purification and Functional Characterization of Factor I
Factor I (FI) is a soluble, 88 kDa glycoprotein present in plasma at a concentration of approximately 35 mg/L. FI inhibits all complement pathways as it degrades activated C4b and C3b when these are bound to a...
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Protocol
Purification and Functional Characterization of C4b-Binding Protein (C4BP)
C4b-binding protein (C4BP) is a soluble, 570 kDa large glycoprotein, present in plasma at a concentration of approximately 200 mg/L. C4BP is the main inhibitor of the classical and lectin pathways of complemen...
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Article
Open AccessCartilage oligomeric matrix protein-induced complement activation in systemic sclerosis
Complexes between cartilage oligomeric matrix protein (COMP) and the complement activation product C3b have been found in the circulation of patients with rheumatoid arthritis and systemic lupus erythematosus....
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Article
Killing of CLL and NHL cells by rituximab and ofatumumab under limited availability of complement
Rituximab and ofatumumab are anti-CD20 antibodies applicable to treatment for non-Hodgkin’s lymphoma and chronic lymphocytic leukemia (CLL). Effectiveness of both immunotherapeutics may depend on exhaustible c...
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Article
Open AccessDegradation of neutrophil extracellular traps co-varies with disease activity in patients with systemic lupus erythematosus
The ability to degrade neutrophil extracellular traps (NETs) is reduced in a subset of patients with systemic lupus erythematosus (SLE). NETs consist of chromatin covered with antimicrobial enzymes and are nor...
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Article
A functional variant in the CFI gene confers a high risk of age-related macular degeneration
Johannes van de Ven and colleagues report a rare missense mutation in CFI encoding a p.Gly119Arg substitution that confers a high risk of AMD. Sera from AMD cases carrying the p.Gly119Arg variant mediate the degr...
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Article
Open AccessFactor H autoantibodies and deletion of Complement Factor H-Related protein-1 in rheumatic diseases in comparison to atypical hemolytic uremic syndrome
Complement activation is involved in rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and atypical hemolytic uremic syndrome (aHUS). Autoantibodies to complement inhibitor factor H (FH), particula...
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Article
Open AccessSerum COMP-C3b complexes in rheumatic diseases and relation to anti-TNF-α treatment
Cartilage oligomeric matrix protein (COMP) is found at elevated concentrations in sera of patients with joint diseases such as rheumatoid arthritis (RA) and osteoarthritis (OA). We recently showed that COMP ac...
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Article
Open AccessMutations in genes encoding complement inhibitors CD46 and CFHaffect the age at nephritis onset in patients with systemic lupus erythematosus
Inherited deficiencies of several complement components strongly predispose to systemic lupus erythematosus (SLE) while deficiencies of complement inhibitors are found in kidney diseases such as atypical hemol...
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Article
Hypoxia increases susceptibility of non-small cell lung cancer cells to complement attack
The complement system can be specifically targeted to tumor cells due to molecular changes on their surfaces that are recognized by complement directly or via naturally occurring antibodies. However, tumor cel...
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Chapter and Conference Paper
Viral Heparin-Binding Complement Inhibitors – A Recurring Theme
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Article
Plasma bikunin: Half-life and tissue uptake
Bikunin is a chondroitin sulfate-containing plasma protein synthesized in the liver. In vitro, it has been shown to inhibit proteases and to have additional activities, but its biological function is still unclea...
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Article
Screening the molecular surface of human anticoagulant protein C: A search for interaction sites
Protein C (PC), a 62 kDa multi-modular zymogen, is activated to an anticoagulant serine protease (activated PC or APC) by thrombin bound to thrombomodulin on the surface of endothelial cells. PC/APC interacts ...
