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Encyclopedia of Medical Immunology pp 1–8Cite as

C5b-C9 Deficiency

  • Living reference work entry
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Synonyms

LCCD: Late component complement deficiency

MAC: Membrane attack complex

TCC: Terminal complement complex

TP: Terminal pathway

Complement System, C5, C6, C7, C8, C9, component

Definition

C5b-C9 represents the terminal pathway of complement system. Complete genetic deficiency of any of those components can lead to increased risk of meningococcal disease, often recurrent infections caused by unusual serogroups.

C5b-9

Three major activation pathways, the classical, the alternative, and the lectin pathway, lead to a common pathway, the terminal complement system that consists of components five to nine. All pathways lead to cleavage of C3, and when additional C3b molecules are bound to the C3 convertases, this results in formation of C5 convertases (C4b2a3b) and subsequently cleavage of C5. This splits C5 into the small C5a molecule, and the larger C5b molecule, which is the initial component of the membrane attack complex (MAC). C5a is a strong chemotactic factor and an...

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Authors and Affiliations

  1. Faculdade de Medicina ABC, Santo Andre, SP, Brazil

    Anete Sevciovic Grumach

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  1. Anete Sevciovic Grumach
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Correspondence to Anete Sevciovic Grumach .

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Editors and Affiliations

  1. Molecular Biology, Monash University Dept. Biochemistry &, Clayton, Victoria, Australia

    Ian MacKay

  2. Autoimmune Disease Research Center, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

    Noel R. Rose

Section Editor information

  1. University of Pennsylvania, PA 19104, Philadelphia, USA

    Kathleen Sullivan

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Grumach, A.S. (2016). C5b-C9 Deficiency. In: MacKay, I., Rose, N. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9209-2_3-1

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  • DOI: https://doi.org/10.1007/978-1-4614-9209-2_3-1

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