Endothelial-derived complement factor D contributes to endothelial dysfunction in malignant nephrosclerosis via local complement activation.

ABSTRACT: Malignant nephrosclerosis is a thrombotic microangiopathy associated with abnormal local activation of the complement alternative pathway (AP). However, the mechanism underlying local AP activation is not fully understood. We hypothesized that complement factor D (CFD) secreted by endothelial cells triggers vascular dysfunction in malignant nephrosclerosis via local complement activation. We investigated the deposition of CFD in human kidney biopsy tissues and the function of endothelial-derived CFD in endothelial cell cultures. Immunofluorescence microscopy and laser microdissection-targeted mass spectrometry revealed significant deposition of CFD in the kidneys of patients with malignant nephrosclerosis. Conditionally immortalized human glomerular endothelial cells (CiGEnCs) continuously

SUBMITTER: Wang Z 

PROVIDER: S-EPMC10184087 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature