ArticlePhysiological genomics2012
Role of genetic modifiers in an orthologous rat model of ARPKD.
Article in Physiological genomics, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 26 citations in OpenAlex.
- Pkhd1Journal of molecular medicine (Berlin, Germany) · 2023Article
- Predictors of progression in autosomal dominant and autosomal recessive polycystic kidney disease.Pediatric nephrology (Berlin, Germany) · 2021Review
- Is There a Functional Role of Mitochondrial Dysfunction in the Pathogenesis of ARPKD?Frontiers in medicine · 2021Article
- Renal cyst growth is attenuated by a combination treatment of tolvaptan and pioglitazone, while pioglitazone treatment alone is not effective.Scientific reports · 2020Article
- Novel compound heterozygous PKHD1 mutations cause autosomal recessive polycystic kidney disease in a Han Chinese family.Molecular medicine reports · 2019Article
- Knockout ofAmerican journal of physiology. Renal physiology · 2019Article
- Atmin modulates Pkhd1 expression and may mediate Autosomal Recessive Polycystic Kidney Disease (ARPKD) through altered non-canonical Wnt/Planar Cell Polarity (PCP) signalling.Biochimica et biophysica acta. Molecular basis of disease · 2019Article
- Impairment of Wnt11 function leads to kidney tubular abnormalities and secondary glomerular cystogenesis.BMC developmental biology · 2016Article
- Magnetic resonance microscopy of renal and biliary abnormalities in excised tissues from a mouse model of autosomal recessive polycystic kidney disease.Physiological reports · 2015Article
- Clinical and genetic characterization of a founder PKHD1 mutation in Afrikaners with ARPKD.Pediatric nephrology (Berlin, Germany) · 2015Article
- Improved Structure and Function in Autosomal Recessive Polycystic Rat Kidneys with Renal Tubular Cell Therapy.PloS one · 2015Article
- Genetic and phenotypic characterization of a Japanese wild-derived DOB/Oda rat strain.Mammalian genome : official journal of the International Mammalian Genome Society · 2013Article
- Genetic background of nonmutant Piebald-Virol-Glaxo rats does not influence nephronophthisis phenotypes.International journal of nephrology and renovascular disease · 2013Article
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8 authors at 1 institution in 1 country.
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Abstract
Human data and animal models of autosomal recessive polycystic kidney disease (ARPKD) suggest that genetic factors modulate the onset and severity of the disease. We report here for the first time that ARPKD susceptibility is attenuated by introgressing the mutated Pkhd1 disease allele from the polycystic kidney (PCK) rat onto the FHH (Fawn-Hooded Hypertensive) genetic background. Compared with PCK, the FHH.Pkhd1 strain had significantly decreased renal cyst formation that coincided with a threefold reduction in mean kidney weights. Further analysis revealed that the FHH. Pkhd1 is protected from increased blood pressure as well as elevated plasma creatinine and blood urea nitrogen levels. On the other hand, liver weight and biliary cystogenesis revealed no differences between PCK and FHH.Pkdh1, indicating that genes within the FHH genetic background prevent the development of renal, but not hepatic, manifestations of ARPKD. Microarray expression analysis of kidneys from 30-day-old PCK rats revealed increased expression of genes previously identified in PKD renal expression profiles, such as inflammatory response, extracellular matrix synthesis, and cell proliferation genes among others, whereas the FHH.Pkhd1 did not show activation of these common markers of disease. This newly developed strain can serve as a tool to map modifier genes for renal disease in ARPKD and provides further insight into disease variability and pathophysiology.
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