The Role of von Willebrand Factor in the Pathogenesis of C3 Glomerulopathy.

ABSTRACT:

Introduction

C3 glomerulopathy (C3G) is caused by dysregulation of complement activation. Our previous study found that patients with C3G showed rare variants in von Willebrand factor (vWF). However, the role of vWF in the pathogenesis of C3G is still unclear.

Method

Patients with C3G diagnosed using kidney biopsy in Peking University First Hospital from 2012 to 2021 were included in the study. The levels of vWF in plasma, urine, and kidney specimens of patients with C3G were detected using enzyme-linked immunosorbent assay (ELISA) or immunofluorescence, respectively, and their associations with clinicopathological data were analyzed. VWF-knockout C3G (FH ^m/m P ^-/- VWF ^m/m ) mice were generated

SUBMITTER: Hua Z 

PROVIDER: S-EPMC12231005 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature