Potentiation of complement regulator factor H protects human endothelial cells from complement attack in aHUS sera.

ABSTRACT: Mutations in the gene encoding for complement regulator factor H (FH) severely disrupt its normal function to protect human cells from unwanted complement activation, resulting in diseases such as atypical hemolytic uremic syndrome (aHUS). aHUS presents with severe hemolytic anemia, thrombocytopenia, and renal disease, leading to end-stage renal failure. Treatment of severe complement-mediated disease, such as aHUS, by inhibiting the terminal complement pathway, has proven to be successful but at the same time fails to preserve the protective role of complement against pathogens. To improve complement regulation on human cells without interfering with antimicrobial activity, we identified an anti-FH monoclonal antibody (mAb) that induced increased FH-mediated protection of primary human en

SUBMITTER: Pouw RB 

PROVIDER: S-EPMC6391659 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature