The atypical hemolytic uremic syndrome (aHUS) is a paradigm of a disease, caused by overactivation of the alternative complement pathway secondary to a not well-understood trigger event. About 60 % of the patients present genetic or acquired abnormalities in the proteins of the alternati ...
Factor H-related proteins (CFHRs) are plasma glycoproteins related in structure and antigenicity to each other and to the complement inhibitory protein factor H. Such proteins are found in most mammals but their number and domain composition vary. This chapter summarizes our current kn ...
Complement Factor H (FH) is an abundant, non-enzymic plasma/serum glycoprotein, which has a major role in regulating activation of the complement system. It can be purified from human plasma/serum by affinity chromatography, using a monoclonal anti-FH antibody as ligand. Other affinity ...
C1 inhibitor is a multipotent serpin capable of inhibiting the classical and the lectin pathways of complement, the fibrinolytic system, and contact/kinin system of coagulation. Deficiency of C1 inhibitor manifest as hereditary angioedema (HAE), an autosomal dominant hereditary ...
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 cofactor such as C4b-binding protein or factor H. Here, we describe a method for purification of FI f ...
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 complement, where it controls C4b-mediated reactions. Here, we describe a method for purifica ...
Properdin is a member of the alternative pathway of complement. It is unique in that it is the only known positive regulator of the complement system. Properdin can stabilize and promote complement activation, but in addition is also capable of initiating the alternative pathway, making it a uni ...
Ficolins constitute a group of lectins involved in innate immunity. L-Ficolin, H-ficolin, and M-ficolin are present in human serum. The human ficolins differ in carbohydrate-binding specificity, but they have in common the ability to recognize the acetyl group. L-Ficolin and H-ficolin are ...
Mannan-binding lectin (MBL) is a soluble pattern recognition molecule of the innate immune system. It is found in plasma in complex with MBL-associated serine proteases (MASPs). When MBL recognizes foreign, e.g., the surface of some microorganisms, or altered host surfaces the MASPs are act ...
Pyrosequencing represents one of the most thorough methods used to analyze polymorphisms. One advantage of using pyrosequencing for genotyping is the ability to identify not only single-nucleotide polymorphisms (SNPs) but also tri-allelic variations, insertions and deleti ...
In order to comprehensively manipulate the human proteome we require a vast repertoire of pharmacological reagents. To address these needs we have developed repertoires of synthetic antibodies by phage display, where diversified oligonucleotides are used to modify the compleme ...
Here we describe a detailed protocol for the one-step preparation of antigen-specific human chimeric immunoglobulin G (IgG) monoclonal antibodies (mAbs) using an in vitro antibody design method referred to as the ADLib (Autonomously Diversifying Library) system. This method empl ...
Antibody humanization is an essential technology for reducing the potential risk of immunogenicity associated with animal-derived antibodies and has been applied to a majority of the therapeutic antibodies on the market. For developing an antibody molecule as a pharmaceutical at t ...
Epstein–Barr virus (EBV) is a herpes virus which in vitro efficiently immortalizes nearly all human B lymphocytes. The lymphoblastoid diploid cell lines (LCL’s) thus generated preserve the characteristics of the cells initially infected by the virus: the cells produce and secrete immu ...
Immunoglobulins (Ig) or antibodies are heavy plasma proteins, with sugar chains added to amino-acid residues by N-linked glycosylation and occasionally by O-linked glycosylation. The versatility of antibodies is demonstrated by the various functions that they mediate such as neu ...
There are over 30 monoclonal antibodies that are FDA approved for a variety of diseases ranging from malignancies to autoimmune diseases to macular degeneration. These antibodies include murine, fully humanized, and chimeric antibodies. There are a number of monoclonal antibodies u ...
Monoclonal antibodies (mAbs) are antibodies of a single antigen specificity produced by identical immune cells, i.e., clones of a common germ cell. They offer unprecedented opportunities to drug development because of their ability to target almost any cell surface or secreted molecule ...
In the broadest sense there are no longer any technical limitations to making human mAbs. Biological issues involving the type and nature of either a synthetic or a natural antibody, advantages of various B cell immunological compartments, and various assays needed to qualitate and quanti ...
One of the major reasons for seeking human monoclonal antibodies has been to eliminate immunogenicity seen with rodent antibodies. Thus far, there has yet been no approach which absolutely abolishes that risk for cell-binding antibodies. In this short article, I draw attention to classic ...
Intravenous immunoglobulin (IVIG) is used successfully for therapy of inflammatory and autoimmune diseases, especially in cases of conventional therapy resistance. Within the broad spectrum of immunomodulatory activities of IVIG in vitro and in vivo, the anti-idiotypic acti ...