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Article

The ciliopathy disease protein NPHP9 promotes nuclear delivery and activation of the oncogenic transcriptional regulator TAZ.

Human molecular genetics - 15 Dec 2012

Habbig Sandra, Bartram Malte P, Sägmüller Josef G, Griessmann Anabel, Franke Mareike, Müller Roman-Ulrich, Schwarz Ricarda, Hoehne Martin, Bergmann Carsten, Tessmer Claudia, Reinhardt H Christian, Burst Volker, Benzing Thomas, Schermer Bernhard

Abstract excerpt

Nephronophthisis (NPH) is a genetically heterogenous kidney disease and represents the most common genetic cause for end-stage renal disease in children. It is caused by the mutation of genes encoding for the nephrocystin proteins (NPHPs) which localize to primary cilia or centrosomes, classifying this disease as a 'ciliopathy'. Recently, it has been shown that NPHP4 acts as a potent negative regulator of...

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Source

Identifiers

PMID: 23026745
DOI: 10.1093/hmg/dds408

Genes

NPHP4
NEK8

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

The ciliopathy disease protein NPHP9 promotes nuclear delivery and activation of the oncogenic transcriptional regulator TAZ.PMID 23026745Read ↗

Mutations in NEK8 link multiple organ dysplasia with altered Hippo signalling and increased c-MYC expression.Semantic proximity Read ↗

Nephrocystin-4 regulates Pyk2-induced tyrosine phosphorylation of nephrocystin-1 to control targeting to monocilia.Semantic proximity Read ↗

The Nek8 protein kinase, mutated in the human cystic kidney disease nephronophthisis, is both activated and degraded during ciliogenesis.Semantic proximity Read ↗

Anks3 alters the sub-cellular localization of the Nek7 kinase.Semantic proximity Read ↗

YAP restricts renal inflammation and mitigates kidney damage in nephronothisis related kidney diseaseSemantic proximity Read ↗

Nephrocystin-1 and nephrocystin-4 are required for epithelial morphogenesis and associate with PALS1/PATJ and Par6Semantic proximity Read ↗

SUMOylation Blocks the Ubiquitin-Mediated Degradation of the Nephronophthisis Gene Product Glis2/NPHP7.Semantic proximity Read ↗

Characterization of the nephrocystin/nephrocystin-4 complex and subcellular localization of nephrocystin-4 to primary cilia and centrosomes.Semantic proximity Read ↗

NEK8 links the ATR-regulated replication stress response and S phase CDK activity to renal ciliopathies.Semantic proximity Read ↗

The polyomavirus middle T-antigen oncogene activates the Hippo pathway tumor suppressor Lats in a Src-dependent manner.Semantic proximity Read ↗

The ciliary protein Nek8/Nphp9 acts downstream of Inv/Nphp2 during pronephros morphogenesis and left-right establishment in zebrafish.Semantic proximity Read ↗

Defects in ciliary localization of Nek8 is associated with cystogenesis.Semantic proximity Read ↗

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