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slit diaphragm
Wednesday 7 March 2007
The slit diaphragm is a specialized cell junction found in the kidney.
Nephrin (NPHS1), Neph1 (KIRREL) and podocin (NPHS2) seem to form a multifunctional receptor complex at the slit diaphragm. Most of the other components of the slit diaphragm interact directly with this complex, in many cases coupling slit diaphragm components to the podocyte’s actin cytoskeleton.
Slit diaphragm-associated proteins
nephrin (NPHS1)
podocin (NPHS2)
alpha-actinin-4
CD2-associated protein
transient receptor potential channel 6
Pathology
A growing number of molecules that function at the slit diaphragm 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