ArticleAmerican journal of physiology. Renal physiology2014
Tissue-specific regulation of the mouse Pkhd1 (ARPKD) gene promoter.
Article in American journal of physiology. Renal physiology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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23 citing papers in PubMed, 42 citations in OpenAlex.
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- Expression of active B-Raf proto-oncogene in kidney collecting ducts induces cyst formation in normal mice and accelerates cyst growth in mice with polycystic kidney disease.Kidney international · 2022Article
- Multiomics analysis reveals that hepatocyte nuclear factor 1β regulates axon guidance genes in the developing mouse kidney.Scientific reports · 2022Article
- A polycystin-2 protein with modified channel properties leads to an increased diameter of renal tubules and to renal cysts.Journal of cell science · 2021Article
- Regulation of polycystin expression, maturation and trafficking.Cellular signalling · 2020Review
- Interstitial microRNA miR-214 attenuates inflammation and polycystic kidney disease progression.JCI insight · 2020Article
- Metalloproteinase PAPP-A regulation of IGF-1 contributes to polycystic kidney disease pathogenesis.JCI insight · 2020Article
- New insights into the role of HNF-1β in kidney (patho)physiology.Pediatric nephrology (Berlin, Germany) · 2019Review
- Mechanism of Fibrosis inJournal of the American Society of Nephrology : JASN · 2018Article
- Comprehensive genetic testing in children with a clinical diagnosis of ARPKD identifies phenocopies.Pediatric nephrology (Berlin, Germany) · 2018Article
- Lkb1 deficiency confers glutamine dependency in polycystic kidney disease.Nature communications · 2018Article
- Suppression of microRNA Activity in Kidney Collecting Ducts Induces Partial Loss of Epithelial Phenotype and Renal Fibrosis.Journal of the American Society of Nephrology : JASN · 2018Article
- Adenylyl cyclase 5 deficiency reduces renal cyclic AMP and cyst growth in an orthologous mouse model of polycystic kidney disease.Kidney international · 2018Article
- Hepatocyte Nuclear Factor-1Journal of the American Society of Nephrology : JASN · 2017Article
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Authors and funding
8 authors at 1 institution in 1 country.
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Abstract
Autosomal recessive polycystic kidney disease, an inherited disorder characterized by the formation of cysts in renal collecting ducts and biliary dysgenesis, is caused by mutations of the polycystic kidney and hepatic disease 1 (PKHD1) gene. Expression of PKHD1 is tissue specific and developmentally regulated. Here, we show that a 2.0-kb genomic fragment containing the proximal promoter of mouse Pkhd1 directs tissue-specific expression of a lacZ reporter gene in transgenic mice. LacZ is expressed in renal collecting ducts beginning during embryonic development but is not expressed in extrarenal tissues. The Pkhd1 promoter contains a binding site for the transcription factor hepatocyte nuclear factor (HNF)-1β, which is required for activity in transfected cells. Mutation of the HNF-1β-binding site abolishes the expression of the lacZ reporter gene in renal collecting ducts. Transgenes containing the 2.0-kb promoter and 2.7 kb of additional genomic sequence extending downstream to the second exon are expressed in the kidney, intrahepatic bile ducts, and male reproductive tract. This pattern overlaps with the endogenous expression of Pkhd1 and coincides with sites of expression of HNF-1β. We conclude that the proximal 2.0-kb promoter is sufficient for tissue-specific expression of Pkhd1 in renal collecting ducts in vivo and that HNF-1β is required for Pkhd1 promoter activity in collecting ducts. Additional genomic sequences located from exons 1-2 or elsewhere in the gene locus are required for expression in extrarenal tissues.
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