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Article

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

International journal of molecular sciences - 15 Mar 2026

Du Xuanjin, Wang Chunyan, Fang Ye, Wang Gangqi, Zhai Yihui, Shen Qian, Tang Xiaoshan, Xu Hong

Abstract excerpt

Patients with nephronophthisis caused by nephrocystin 3 (NPHP3) variants rapidly progress to end-stage kidney disease. However, existing Nphp3 mouse models fail to fully recapitulate the characteristics of this disease. We generated a renal tubule-specific Nphp3 knockout mouse model that more accurately mirrors the human disease course. The mouse model was first validated by confirming the loss of Nphp3 protein...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 41898549
DOI: 10.3390/ijms27062687
PMCID: PMC13026956

Genes

NPHP3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

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