ArticlePloS one2013
A telomerase immortalized human proximal tubule cell line with a truncation mutation (Q4004X) in polycystin-1.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 15 citations in OpenAlex.
- Eliosin-an alternative product from the HmPKD1 locus is a component of endoplasmic reticulum mitochondria membrane contact sites.PloS one · 2025Article
- The chromatin landscape of healthy and injured cell types in the human kidney.Nature communications · 2024Article
- Polycystin-1 Interacting Protein-1 (CU062) Interacts with the Ectodomain of Polycystin-1 (PC1).Cells · 2023Article
- Fundamental insights into autosomal dominant polycystic kidney disease from human-based cell models.Pediatric nephrology (Berlin, Germany) · 2019Article
- Expression and secretion of CXCL12 are enhanced in autosomal dominant polycystic kidney disease.BMB reports · 2019Article
- Rifampin modulation of xeno- and endobiotic conjugating enzyme mRNA expression and associated microRNAs in human hepatocytes.Pharmacology research & perspectives · 2018Article
- Genetic Variants Contributing to Colistin Cytotoxicity: Identification of TGIF1 and HOXD10 Using a Population Genomics Approach.International journal of molecular sciences · 2017Article
- Transcriptome analysis reveals manifold mechanisms of cyst development in ADPKD.Human genomics · 2016Article
- Virtual-tissue computer simulations define the roles of cell adhesion and proliferation in the onset of kidney cystic disease.Molecular biology of the cell · 2016Article
- Autocrine IL-10 activation of the STAT3 pathway is required for pathological macrophage differentiation in polycystic kidney disease.Disease models & mechanisms · 2016Article
- Rifampin Regulation of Drug Transporters Gene Expression and the Association of MicroRNAs in Human Hepatocytes.Frontiers in pharmacology · 2016Article
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Authors and funding
14 authors at 5 institutions in 1 country.
Funding
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is associated with a variety of cellular phenotypes in renal epithelial cells. Cystic epithelia are secretory as opposed to absorptive, have higher proliferation rates in cell culture and have some characteristics of epithelial to mesenchymal transitions. In this communication we describe a telomerase immortalized cell line that expresses proximal tubule markers and is derived from renal cysts of an ADPKD kidney. These cells have a single detectable truncating mutation (Q4004X) in polycystin-1. These cells make normal appearing but shorter cilia and fail to assemble polycystin-1 in the cilia, and less uncleaved polycystin-1 in membrane fractions. This cell line has been maintained in continuous passage for over 35 passages without going into senescence. Nephron segment specific markers suggest a proximal tubule origin for these cells and the cell line will be useful to study mechanistic details of cyst formation in proximal tubule cells.
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