Of mice and men: The factor H protein family and complement regulation.

Abstract:

:For decades immunological research has relied, with variable success, on mouse models to investigate diseases and possible therapeutic interventions. With the approval of the first therapeutic antibody targeting complement, called eculizumab, as therapy in paroxysmal nocturnal hemoglobinuria (PNH) and more recently atypical hemolytic uremic syndrome (aHUS), the viability of targeting the complement system was demonstrated. The potent, endogenous complement regulators have become of increasing interest as templates for designing and developing new therapeutics. Recently, complement inhibitors based on (parts of) the human complement regulator factor H (FH) are being examined for therapeutic intervention in inflammatory conditions. The first step to evaluate the potency of a new drug is often testing it in a mouse model for the target disease. However, translating results to human conditions requires a good understanding of similarities and, more importantly, differences between the human and mouse complement system and particularly regulation. This review will provide a comprehensive overview of the complement regulator FH and its closely related proteins and current views on their role in mice and men.

journal_name

Mol Immunol

journal_title

Molecular immunology

authors

Pouw RB,Vredevoogd DW,Kuijpers TW,Wouters D

doi

10.1016/j.molimm.2015.03.011

subject

Has Abstract

pub_date

2015-09-01 00:00:00

pages

12-20

issue

1

eissn

0161-5890

issn

1872-9142

pii

S0161-5890(15)00115-7

journal_volume

67

pub_type

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