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Complement Regulators in Human Disease: Lessons From Modern Genetics

Complement Regulators in Human Disease: Lessons From Modern Genetics

M K Liszewski, J P Atkinson

J Intern Med. 2015 Mar;277(3):294-305.

PMID: 25495259

Abstract:

First identified in human serum in the late 19th century as a 'complement' to antibodies in mediating bacterial lysis, the complement system emerged more than a billion years ago probably as the first humoral immune system. The contemporary complement system consists of nearly 60 proteins in three activation pathways (classical, alternative and lectin) and a terminal cytolytic pathway common to all. Modern molecular biology and genetics have not only led to further elucidation of the structure of complement system components, but have also revealed function-altering rare variants and common polymorphisms, particularly in regulators of the alternative pathway, that predispose to human disease by creating 'hyperinflammatory complement phenotypes'. To treat these 'complementopathies', a monoclonal antibody against the initiator of the membrane attack complex, C5, has received approval for use. Additional therapeutic reagents are on the horizon.

Chemicals Related in the Paper:

Catalog Number	Product Name	Structure	CAS Number	Price
AP80295530	Complement C5 from human serum		80295-53-0	Price

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