Podocyte-Specific Sialylation-Deficient Mice Serve as a Model for Human FSGS.

ABSTRACT:

Background

The etiology of steroid-resistant nephrotic syndrome, which manifests as FSGS, is not completely understood. Aberrant glycosylation is an often underestimated factor for pathologic processes, and structural changes in the glomerular endothelial glycocalyx have been correlated with models of nephrotic syndrome. Glycans are frequently capped by sialic acid (Sia), and sialylation's crucial role for kidney function is well known. Human podocytes are highly sialylated; however, sialylation's role in podocyte homeostasis remains unclear.

Methods

We generated a podocyte-specific sialylation-deficient mouse model (PCmas^-/- ) by targeting CMP-Sia synthetase, and used histologic and ultrastructural analysis to decipher the phenotype. We applied CRISPR/Cas9

SUBMITTER: Niculovic KM 

PROVIDER: S-EPMC6551790 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature