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Article

Podocin oligomers regulate the ordering of nephrin chains, providing the molecular basis of NPHS2 interallelic interactions.

Proceedings of the National Academy of Sciences of the United States of America - 21 Apr 2026

Antal-Kónya Violetta, Schay Gusztáv, Kétszeri Máté, Balogh Eszter, Mikó Ágnes, Seidl Dániel, Keszthelyi Tália Magdolna, Bernáth Mária, Ungvári-Veres Anita, Dorval Guillaume, Saleem Moin A, Dixit Abhijit, Antignac Corinne, Pászty Katalin, Kellermayer Miklós, Menyhárd Dóra K, Tory Kálmán

Abstract excerpt

The most frequent causative genes in podocytopathies are NPHS1 and NPHS2 that encode the main slit diaphragm components nephrin and podocin, respectively. The significance of the nephrin-podocin interaction has remained elusive. The NPHS2 R229Q variant is unique in human genetics as it is only pathogenic when trans-associated to specific 3' variants causing R229Q podocin to form distorted heterooligomers. To...

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Identifiers

PMID: 41980097
DOI: 10.1073/pnas.2524893123
PMCID: PMC13099668

Genes

NPHS2
NPHS1

Variants

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Podocin oligomers regulate the ordering of nephrin chains, providing the molecular basis of NPHS2 interallelic interactions.PMID 41980097Read ↗

Molecular basis of the functional podocin-nephrin complex: mutations in the NPHS2 gene disrupt nephrin targeting to lipid raft microdomains.Semantic proximity Read ↗

In silico Structural characterization of podocin and assessment of nephrotic syndrome-associated podocin mutants.Semantic proximity Read ↗

Structural conformations of intrinsically disordered proteins of podocyte slit-diaphragmSemantic proximity Read ↗

NPHS2 (Podocin) mutations in nephrotic syndrome. Clinical spectrum and fine mechanisms.Semantic proximity Read ↗

Kidney Organoids Generated Using an Allelic Series of NPHS2 Point Variants Reveal Distinct Intracellular Podocin Mistrafficking.Semantic proximity Read ↗

Co-Inheritance of Functional Podocin Variants with Heterozygous Collagen IV Mutations Predisposes to Renal Failure.Semantic proximity Read ↗

CD2AP’s Structure and Oligomerization are compromised by the K301M mutation: implications for Nephrotic syndromeSemantic proximity Read ↗

The relationship among nephrin, podocin, CD2AP, and alpha-actinin might not be a true 'interaction' in podocyte.Semantic proximity Read ↗

Dysregulated Dynein-Mediated Trafficking of Nephrin Causes INF2-related Podocytopathy.Semantic proximity Read ↗

Interaction with Podocin Facilitates Nephrin SignalingSemantic proximity Read ↗

Plasma membrane targeting of podocin through the classical exocytic pathway: effect of NPHS2 mutations.Semantic proximity Read ↗

A small molecule chaperone rescues keratin-8 mediated trafficking of misfolded podocin to correct genetic Nephrotic Syndrome.Semantic proximity Read ↗

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