Modeling complement activation on human glomerular microvascular endothelial cells.

ABSTRACT:

Introduction

Atypical hemolytic uremic syndrome (aHUS) is a rare kidney disease caused by dysregulation of the complement alternative pathway. The complement dysregulation specifically leads to damage to the glomerular endothelium. To further understand aHUS pathophysiology, we validated an ex vivo model for measuring complement deposition on both control and patient human glomerular microvascular endothelial cells (GMVECs).

Methods

Endothelial cells were incubated with human test sera and stained with an anti-C5b-9 antibody to visualize and quantify complement depositions on the cells with immunofluorescence microscopy.

Results

First, we showed that zymosan-activated sera resulted in increased endothelial C5b-9 depositions compared to normal human serum (NHS).

SUBMITTER: Stevens KH 

PROVIDER: S-EPMC10634509 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature