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Article

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

FEBS letters - 30 Jul 2012

Fukui Hajime, Shiba Dai, Asakawa Kazuhide, Kawakami Koichi, Yokoyama Takahiko

Abstract excerpt

Nephronophthisis (NPHP) is an autosomal recessive cystic kidney disease. Among 12 reported Nphp gene products, Inv/Nphp2, Nphp3 and Nek8/Nphp9 are localized to the proximal segment in the primary cilium. However, the functional relationships are unknown. This study focused on phenotype analysis of nek8 knockdown embryos and the genetic relationship between nek8 and inv in zebrafish. Knockdown of nek8 produced...

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Identifiers

PMID: 22687244
DOI: 10.1016/j.febslet.2012.05.064

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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.

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The ciliary protein Nek8/Nphp9 acts downstream of Inv/Nphp2 during pronephros morphogenesis and left-right establishment in zebrafish.PMID 22687244Read ↗

Loss of the ciliary kinase Nek8 causes left-right asymmetry defects.Semantic proximity Read ↗

Novel NEK8 Mutations Cause Severe Syndromic Renal Cystic Dysplasia through YAP Dysregulation.Semantic proximity Read ↗

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

NPHP4 variants are associated with pleiotropic heart malformations.Semantic proximity Read ↗

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

Nephrocystin-4 is required for pronephric duct-dependent cloaca formation in zebrafish.Semantic proximity Read ↗

Nephrocystin-3 is required for ciliary function in zebrafish embryos.Semantic proximity Read ↗

Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination.Semantic proximity Read ↗

Dynein axonemal intermediate chain 2 is required for formation of the left-right body axis and kidney in medaka.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 a central component of a nephronophthisis module linking NEK8 to INVS and NPHP3.Semantic proximity Read ↗

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

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