C1 Deficiency and Associated Disorders
Living reference work entry
The complement system is a strictly regulated and highly complex effector system whose major function is to recognize and eliminate pathogens as well as altered self-antigens. Therefore, it constitutes a very powerful arm of the innate and adaptive immune systems with unique ability to discriminate self from nonself and eliminate “danger” through a wide array of processes that include phagocytosis and cytolytic mechanisms. Although there are three interdependent pathways of complement activation – classical, alternative, and lectin – only the role of the classical pathway and the consequences of deficiency in any of the components that initially trigger its activation are discussed here. The first component of complement (Table 1) is a multimolecular complex comprising of one molecule of C1q and the Ca 2+-dependent tetramer – C1r-C1s-C1s-C1r – which give rise to the pentameric complex: C1q.C1r 2.C1s 2found in plasma. Each molecule within this complex plays a sequential and...
- Gaboriaud C, Païdassi H, Frachet P, Tacnet-Delorme P, Thielens NM, Arlaud GJ. C1q: a versatile pattern recognition molecule and sensor of altered self. In: Kilpatrick D, editor. Collagen-related lectins in innate immunity (Research Signpost), vol. 81; 2007. p. 1–15.Google Scholar
- Ghebrehiwet B, Hosszu K, Valentino A, Peerschke EIB. The C1q family of proteins: insights into the emerging non-traditional functions. Front Immunol. 2012;3.pii:52.3:1–9.Google Scholar
- Lahita RG. Systemic lupus erythematosus. Third ed. NY: Academic Press, New York; 1999.Google Scholar
© Springer Science+Business Media LLC 2016