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Article

Kidney-specific inactivation of the Pkd1 gene induces rapid cyst formation in developing kidneys and a slow onset of disease in adult mice

12 Sept 2007

Abstract excerpt

Autosomal dominant polycystic kidney disease, caused by mutations in the PKD1 gene, is characterized by progressive deterioration of kidney function due to the formation of thousands of cysts leading to kidney failure in mid-life or later. How cysts develop and grow is currently unknown, although extensive research revealed a plethora of cellular changes in cyst lining cells. We have constructed a...

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PMID: 17932118
DOI: 10.1093/hmg/ddm299

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Related research

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Kidney-specific inactivation of the Pkd1 gene induces rapid cyst formation in developing kidneys and a slow onset of disease in adult micePMID 17932118Read ↗

Pkd1 inactivation induced in adulthood produces focal cystic disease.Semantic proximity Read ↗

A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1.Semantic proximity Read ↗

A mouse model for polycystic kidney disease through a somatic in-frame deletion in the 5' end of Pkd1.Semantic proximity Read ↗

Scattered Deletion of PKD1 in Kidneys Causes a Cystic Snowball Effect and Recapitulates Polycystic Kidney Disease.Semantic proximity Read ↗

Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling pathwaysSemantic proximity Read ↗

Inactivation of Pkd1 in principal cells causes a more severe cystic kidney disease than in intercalated cells.Semantic proximity Read ↗

Rapamycin-mediated suppression of renal cyst expansion in del34 Pkd1-/- mutant mouse embryos: an investigation of the feasibility of renal cyst prevention in the foetus.Semantic proximity Read ↗

Defining a link with autosomal-dominant polycystic kidney disease in mice with congenitally low expression of Pkd1.Semantic proximity Read ↗

Perinatal lethality with kidney and pancreas defects in mice with a targetted Pkd1 mutation.Semantic proximity Read ↗

Comparison of Pkd1-targeted mutants reveals that loss of polycystin-1 causes cystogenesis and bone defects.Semantic proximity Read ↗

Cyst formation in the PKD2 (1-703) transgenic rat precedes deregulation of proliferation-related pathways.Semantic proximity Read ↗

Pkd1 and Pkd2 Are Required for Normal Placental DevelopmentSemantic proximity Read ↗

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