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Article

Kidney cysts, pancreatic cysts, and biliary disease in a mouse model of autosomal recessive polycystic kidney disease.

Pediatric nephrology (Berlin, Germany) - 1 May 2008

Williams Scott S, Cobo-Stark Patricia, James Leighton R, Somlo Stefan, Igarashi Peter

Abstract excerpt

Mutations in PKHD1 cause autosomal recessive polycystic kidney disease (ARPKD). We produced a mouse model of ARPKD by replacing exons 1-3 of Pkhd1 with a lacZ reporter gene utilizing homologous recombination. This approach yielded heterozygous Pkhd1 (lacZ/+) mice, that expressed beta-galactosidase in tissues where Pkhd1 is normally expressed, and homozygous Pkhd1 (lacZ/lacZ) knockout mice. Heterozygous Pkhd1...

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Source

Identifiers

PMID: 18286309
DOI: 10.1007/s00467-007-0735-4

Genes

PKHD1

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