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Article

The Inv compartment of renal cilia is an intraciliary signal-activating center to phosphorylate ANKS6.

Kidney international - 1 May 2018

Nakajima Yoshiro, Kiyonari Hiroshi, Mukumoto Yoshiko, Yokoyama Takahiko

Abstract excerpt

Connections between cilia and renal cystic diseases are well known, yet molecular mechanisms remain undefined. Cysto-proteins localized in the Inv compartment of cilia (INV, NPHP3, NEK8, and ANKS6) constitute a distinct group. Here we created and analyzed mutant mice (G2A mice) with a defective cilia localization signal in the Nphp3 gene. Mutant NPHP3 was absent the binding capacity of UNC119, a carrier protein...

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Source

Identifiers

PMID: 29395339
DOI: 10.1016/j.kint.2017.11.016

Genes

NEK8
NPHP3

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

The Inv compartment of renal cilia is an intraciliary signal-activating center to phosphorylate ANKS6.PMID 29395339Read ↗

Inv acts as a molecular anchor for Nphp3 and Nek8 in the proximal segment of primary ciliaSemantic proximity Read ↗

ANKS6 is a central component of a nephronophthisis module linking NEK8 to INVS and NPHP3.Semantic proximity Read ↗

Biallelic ANKS6 mutations cause late-onset ciliopathy with chronic kidney disease through YAP dysregulation.Semantic proximity Read ↗

The ANKS3/BICC1 protein complex is a master post-transcriptional regulator of <i>NPHP1</i> ciliopathy-gene transcriptsSemantic proximity Read ↗

Structural basis of the Inv compartment and ciliary abnormalities in Inv/nphp2 mutant mice.Semantic proximity Read ↗

Localization of Inv in a distinctive intraciliary compartment requires the C-terminal ninein-homolog-containing regionSemantic proximity Read ↗

Anks3 interacts with nephronophthisis proteins and is required for normal renal developmentSemantic proximity Read ↗

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

Loss of nephrocystin-3 function can cause embryonic lethality, Meckel-Gruber-like syndrome, situs inversus, and renal-hepatic-pancreatic dysplasia.Semantic proximity Read ↗

ANKS6 is the critical activator of NEK8 kinase in embryonic situs determination and organ patterning.Semantic proximity Read ↗

Inactivation of Invs/Nphp2 in renal epithelial cells drives infantile nephronophthisis like phenotypes in mouse.Semantic proximity Read ↗

ANKS3 Co-Localises with ANKS6 in Mouse Renal Cilia and Is Associated with Vasopressin Signaling and Apoptosis In Vivo in Mice.Semantic proximity Read ↗

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