ArticlePediatric research2015
Impaired epithelial Na+ channel activity contributes to cystogenesis and development of autosomal recessive polycystic kidney disease in PCK rats.
Article in Pediatric research, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 23 citations in OpenAlex.
- Epithelial cell expansion drives cyst progression in genetic models of autosomal recessive polycystic kidney disease.iScience · 2026Article
- Pannexin channels in the kidney.American journal of physiology. Renal physiology · 2025Review
- Electrolyte and metabolite composition of cystic fluid from a rat model of ARPKD.Communications biology · 2025Article
- Dissociation of Hypertension and Renal Damage After Cessation of High-Salt Diet in Dahl Rats.Hypertension (Dallas, Tex. : 1979) · 2024Article
- Defects of renal tubular homeostasis and cystogenesis in theiScience · 2024Article
- Functional role of histamine receptors in the renal cortical collecting duct cells.American journal of physiology. Cell physiology · 2022Article
- Ion channels as a therapeutic target for renal fibrosis.Frontiers in physiology · 2022Review
- Recent advances in understanding ion transport mechanisms in polycystic kidney disease.Clinical science (London, England : 1979) · 2021Review
- Predictors of progression in autosomal dominant and autosomal recessive polycystic kidney disease.Pediatric nephrology (Berlin, Germany) · 2021Review
- Nephron-Specific Disruption of Polycystin-1 Induces Cyclooxygenase-2-Mediated Blood Pressure Reduction Independent of Cystogenesis.Journal of the American Society of Nephrology : JASN · 2020Article
- Knockout ofAmerican journal of physiology. Renal physiology · 2019Article
- Loss of primary cilia increases polycystin-2 and TRPV4 and the appearance of a nonselective cation channel in the mouse cortical collecting duct.American journal of physiology. Renal physiology · 2019Article
- ATP release into ADPKD cysts via pannexin-1/P2X7 channels decreases ENaC activity.Biochemical and biophysical research communications · 2019Article
- Hydrochlorothiazide ameliorates polyuria caused by tolvaptan treatment of polycystic kidney disease in PCK rats.Clinical and experimental nephrology · 2019Article
- Salt-deficient diet exacerbates cystogenesis in ARPKD via epithelial sodium channel (ENaC).EBioMedicine · 2019Article
- Characterization of purinergic receptor expression in ARPKD cystic epithelia.Purinergic signalling · 2018Article
- Lack of Effects of Metformin and AICAR Chronic Infusion on the Development of Hypertension in Dahl Salt-Sensitive Rats.Frontiers in physiology · 2017Article
- Extraoral Taste Receptor Discovery: New Light on Ayurvedic Pharmacology.Evidence-based complementary and alternative medicine : eCAM · 2017Review
- Functional and therapeutic importance of purinergic signaling in polycystic kidney disease.American journal of physiology. Renal physiology · 2016Review
- Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium.Journal of visualized experiments : JoVE · 2015Article
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
backgroundAutosomal recessive polycystic kidney disease is a genetic disorder characterized by the development of renal cysts of tubular epithelial cell origin. Epithelial Na(+) channel (ENaC) is responsible for sodium reabsorption in the aldosterone-sensitive distal nephron. Here, we investigated the ENaC expression and activity in cystic tissue taken from rats with autosomal recessive polycystic kidney disease.
methodsPolycystic kidney (PCK) rats were treated with the selective ENaC inhibitor benzamil given in the drinking water, and after 4 or 12 wk, the severity of morphological malformations in the kidneys was assessed. ENaC and aquaporin-2 expression and ENaC activity were tested with immunohistochemistry and patch-clamp electrophysiology, respectively.
resultsTreatment with benzamil exacerbated development of cysts compared with the vehicle-treated animals. In contrast, the 12 wk of treatment with the loop diuretic furosemide had no effect on cystogenesis. Single-channel patch-clamp analysis revealed that ENaC activity in the freshly isolated cystic epithelium was significantly lower than that in the noncystic collecting ducts isolated from PCK or normal Sprague-Dawley rats. Immunohistochemical analysis confirmed that β-ENaC and aquaporin-2 expressions in cysts are decreased compared with nondilated tubules from PCK rat kidneys.
conclusionWe demonstrated that cystic epithelium exhibits low ENaC activity and this phenomenon can contribute to cyst progression.
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