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NPHSs

Members: NPHS1, NPHS2

Nephrin (NPHS1), Neph1 (KIRREL) and podocin (NPHS2) seem to form a multifunctional receptor complex at the podocyte slit diaphragm.

Most of the other components of the podocyte slit diaphragm interact directly with this complex, in many cases coupling slit diaphragm components to the podocyte’s actin cytoskeleton.

Patrhology

A growing number of molecules that function at the podocyte slit diaphragm (PSD) have been identified in patients with inherited and sporadic nephrotic syndromes. Genetic deletion of nearly all of these molecules results in proteinuria and effacement of foot processes.

References

- Johnstone DB, Holzman LB. Clinical impact of research on the podocyte slit diaphragm. Nat Clin Pract Nephrol. 2006 May;2(5):271-82. PMID: #16932440#