Anti-Nephrin antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Nephrin also known as NPHS1 is a transmembrane protein with a molecular weight of approximately 180 kDa. Researchers have predominantly observed nephrin in the podocytes of the kidney glomerulus. Nephrin plays a mechanical role as part of the slit diaphragm a structure critical for maintaining the filtration barrier of the kidney. Its structure includes multiple immunoglobulin-like domains which facilitate cell signaling and adhesion processes important for podocyte function.

Biological function summary

Nephrin is essential for the regulation of glomerular permeability and podocyte foot process architecture. It serves as a significant component of the slit diaphragm complex working alongside proteins like podocin and CD2AP. This protein complex helps regulate the integrity and selectivity of the glomerular filtration barrier ensuring that blood plasma contents are selectively filtered while preventing the loss of large proteins into urine.

Pathways

Nephrin is integral to the PI3K/Akt signaling pathway influencing cell survival and cytoskeletal organization in podocytes. Nephrin's interaction within this pathway involves related proteins such as GAPDH and PTPRO which impact cellular functions critical to maintaining kidney structure and function. Additionally nephrin participates in the synaptopodin pathway modulating actin dynamics and therefore playing a role in cellular contraction and movement.

Associated diseases and disorders

Nephrin dysfunction is closely related to congenital nephrotic syndrome particularly Finnish-type nephrosis which results in significant proteinuria and kidney failure. Nephrin mutations or aberrant expression can also contribute to other glomerular diseases such as focal segmental glomerulosclerosis (FSGS). In these conditions nephrin's relationship with other proteins like neph1 and podocin becomes disrupted leading to loss of slit diaphragm integrity and impaired renal function.