Humanized C3 Mouse: A Novel Accelerated Model of C3 Glomerulopathy.

ABSTRACT:

Background

C3 glomerulopathy (C3G) is characterized by the alternative-pathway (AP) hyperactivation induced by nephritic factors or complement gene mutations. Mice deficient in complement factor H (CFH) are a classic C3G model, with kidney disease that requires several months to progress to renal failure. Novel C3G models can further contribute to understanding the mechanism behind this disease and developing therapeutic approaches.

Methods

A novel, rapidly progressing, severe, murine model of C3G was developed by replacing the mouse C3 gene with the human C3 homolog using VelociGene technology. Functional, histologic, molecular, and pharmacologic assays characterize the presentation of renal disease and enable useful pharmacologic interventions in the humanize

SUBMITTER: Devalaraja-Narashimha K 

PROVIDER: S-EPMC7894673 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature