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Article

Renal cysts of inv/inv mice resemble early infantile nephronophthisis.

Journal of the American Society of Nephrology : JASN - 1 Jul 2004

Phillips Carrie L, Miller Karen J, Filson Adele J, Nürnberger Jens, Clendenon Jeffrey L, Cook Gregory W, Dunn Kenneth W, Overbeek Paul A, Gattone Vincent H, Bacallao Robert L

Abstract excerpt

Cystic kidney disease has been linked to mutations in the Invs gene in mice with inversion of embryonic turning (inv/inv) and the INVS (NPHP2) gene in infants with nephronophthisis type 2 (NPHP2). The inv mouse model features multiorgan defects including renal cysts, altered left-right laterality...

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Source

Identifiers

PMID: 15213262
DOI: 10.1097/01.asn.0000131520.07008.b3

Genes

INVS

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026. Prepared record publication-15213262-2026-09-01.1. Manifest publication-graph-cohort-2026-09-01.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 1 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Renal cysts of inv/inv mice resemble early infantile nephronophthisis.PMID 15213262Read ↗

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

Renal Tubule-Specific Deletion of Nephrocystin 3 (Nphp3) Causes Infantile Nephronophthisis-like Phenotypes in Mice.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 ↗

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

Inactivation of <i>Nphp2</i> in renal epithelial cells drives infantile nephronophthisis like phenotypes in mouseSemantic proximity Read ↗

Sustained cell proliferation of renal epithelial cells in mice with inv mutation.Semantic proximity Read ↗

Mutations of NPHP2 and NPHP3 in infantile nephronophthisis.Semantic proximity Read ↗

Early presentation of cystic kidneys in a family with a homozygous INVS mutation.Semantic 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 ↗

Cardiopulmonary malformations in the inv/inv mouse.Semantic proximity Read ↗

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

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