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Studies on the genetics of complement proteins were originally initiated by the discovery of complement deficiencies in animals and man. From these studies, it has become apparent that most complement components are polymorphic, some with several tens of different allotypes. The study ...

The purpose of the immune system is to defend the host from constantly changing microbial pathogens. Autoimmune diseases develop as a consequence of the production of antibodies and/or cells that react with self-antigens, and may recmit other effector mechanisms that result in tissue dam ...

Complement (C) deficiencies are not common and laboratory screening for clinical reasons generally involves examining relatively few samples. Nevertheless, because of the range of tests, complete testing may mean carrying out many different assays. In contrast, the determinati ...

Measurement of complement-mediated cytolysis, described in Chapter 5, is the index most frequently used for assessing aspects of complement activation or regulation. However, other consequences of complement activation are probably of more relevance in vivo. Complement plays a m ...

The human complement (C) system is composed of about 20 components in blood plasma (Chapter 1). Of these, many become deposited at sites of complement attack and are detected by immunohistochemical methods. C3b, C4b, and their fragments remain covalently bound on targets and components of the m ...

Activation of the complement system by the classical, mannan binding lectic (MBL) or alternative pathway results in the generation of multiple complement proteolytic cleavage products, recruited from these inactive precursor molecules in a sequentially proceeding cascade ( ...

The complement (C) system consists of a group of 12 soluble plasma proteins that interact with one another in two distinct enzymatic activation cascades (the classical and alternative pathways) and in the nonenzymatic assembly of a cytolytic complex (the membrane attack pathway) (Fig. 1; T ...

Mature T lymphocytes specifically recognize antigens through interaction between MHC/peptide complex and αβ or γδ TCR. Variable region genes created by recombination of variable (V), diversity (D), and joining (J) locus contribute to shaping T cell receptor (TCR) repertoires (1,2). Th ...

Advances in the study of thymic selection have relied heavily on animals that are genetically manipulated either by transgenesis and/or by knock out technology. Besides being costly and time consuming, these methods suffer inherent limitations (reviewed in refs. 1–3). In an effort to first, ...

During the developmental process of T cells in the thymus, immature CD4CD8 double positive thymocytes (DP) are subject to undergo positive selection process to become either CD4 single positive (SP) or CD8-SP mature cells (1–3). Since both CD4-SP and CD8-SP mature T cells are the progeny of immatu ...

Most T lymphocytes of the immune system differentiate within the thymus along the CD4/CD8 developmental pathway by a highly ordered process termed thymic selection (1,2). The maturation status of thymocytes is commonly assessed by their expression of the coreceptor proteins CD4 and CD8 a ...

Most T cells develop in the thymus. Thymic development of T cells offers an excellent experimental system to pursue many issues of developmental biology, including 1. lin

Gene indices with many thousands of entries have been constructed by tag sequencing of randomly selected cDNA clones (1–7) and are widely available in repositories, such as the dbEST database (16). As more and more genes are identified, efforts are redirected towards understanding the contr ...

Thymically derived lymphocytes perceive antigenic signals via the T-cell antigen receptor-CD3 molecular complex (1,2). The TCR/CD3 is a multimeric complex of proteins composed of an α/β heterodimer that is responsible for the recognition of the antigenic signal (in the case of T cells a pep ...

An increasing number of transmembrane proteins are being identified as substrates for ubiquitination. Ubiquitin is a highly conserved 76 amino acid polypeptide that is covalently added to proteins as monomers or chains as a consequence of the formation of an isopeptide linkage between ...

Cytotoxic T lymphocytes (CTL) play an important role in the control of intracellular pathogens through their capacity to lyse infected target cells or secrete immunostimulatory cytokines, such as IFN-γ or TNF-α (1–3). These features of cytotoxic T cells are termed “effector” mechanisms a ...

The integrin supergene family of adhesion molecules are cell surface αβ heterodimers (reviewed in ref. 1). Although most integrins are involved in the binding of cells to extracellular matrix (ECM) components, many T-cell integrins can also bind to cell surface ligands. CD4+ T helper (Th) cells ...

The size and shape of an individual T lymphocyte can reflect activation or functional status. Therefore, information from a visual assessment of morphology can provide important criteria for determining extrinsic factors that regulate the physiological responses of lymphocyt ...

The earliest steps along the pathway leading to mature T cells in mouse thymus have been defined (1,2). Within the thymus, several minute but discrete populations of T cell precursors develop in sequence, preceding the stage of CD4+8+ thymocytes (Fig. 1). The earliest identifiable intrathymic p ...

T-cell activation by antigen-receptor ligation results in rapid activation of phospholipase-Cγ1 (PLC). Activated PLC hydrolyzes membrane inositol phospholipids (PIP2) generating two second messengers: inositol(1,4,5)tris phosphate (IP3) and diacylglycerol (DG) (1). I ...