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Article

A mouse model of autosomal recessive polycystic kidney disease with biliary duct and proximal tubule dilatation.

Kidney international - 1 Aug 2007

Woollard J R, Punyashtiti R, Richardson S, Masyuk T V, Whelan S, Huang B Q, Lager D J, vanDeursen J, Torres V E, Gattone V H, LaRusso N F, Harris P C, Ward C J

Abstract excerpt

Autosomal recessive polycystic kidney disease (ARPKD) is caused by mutations in the polycystic kidney and hepatic disease (PKHD1) gene encoding the protein fibrocystin/polyductin. The aim of our study was to produce a mouse model of ARPKD in which there was no functional fibrocystin/polyductin to study the pathophysiology of cystic and fibrocystic disease in renal and non-renal tissues. Exon 2 of the gene was...

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Source

Identifiers

PMID: 17519956
DOI: 10.1038/sj.ki.5002294

Genes

PKHD1

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.

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