Nephrin protein (Nephrin)

Nephrin protein is a large transmembrane cell adhesion molecule expressed predominantly on the surface of podocytes in the glomeruli of the kidney. It forms a crucial part of the slit diaphragm, a specialized junction that functions as both a physical and signaling barrier, preventing leakage of plasma proteins into urine and maintaining kidney filtration selectivity. Nephrin consists of multiple extracellular immunoglobulin-like domains and a cytoplasmic tail with conserved tyrosine residues that interact with scaffolding and signaling proteins, such as MAGI1 and Nck, to regulate podocyte structure and signaling. Mutations in the NPHS1 gene encoding nephrin disrupt its structural integrity or signaling function, resulting in congenital nephrotic syndrome (especially the Finnish type) and other severe proteinuric kidney diseases. Research continues into how nephrin's assembly at the slit diaphragm underpins renal health and how experimental agents may modify nephrin-related signaling in disease models.

Other names

NephrinNPHS1 protein

Not applicable for approved drugs; experimental mechanism includes blocking nephrin/MAGI1 signaling complex in the slit diaphragm