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Review
. 2015 Mar;277(3):294-305.
doi: 10.1111/joim.12338. Epub 2015 Jan 16.

Complement regulators in human disease: lessons from modern genetics

M K Liszewski  1 , J P Atkinson  1
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Review

Complement regulators in human disease: lessons from modern genetics

"V体育官网" M K Liszewski et al. J Intern Med. 2015 Mar.
Free article

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 VSports手机版. 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. .

Keywords: age-related macular degeneration; atypical haemolytic uraemic syndrome; complement regulation; eculizumab; genetics; therapeutics V体育安卓版. .

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