ReviewClinical and experimental nephrology2013
Autosomal dominant polycystic kidney disease: recent advances in pathogenesis and potential therapies.
Review in Clinical and experimental nephrology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
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Who cites it
38 citing papers in PubMed, 66 citations in OpenAlex.
- Mammalian target of rapamycin inhibition impacts energy homeostasis and induces sex-specific body weight loss in humans.Journal of cachexia, sarcopenia and muscle · 2023Trial
- The effect of tolvaptan on autosomal dominant polycystic kidney disease patients: a subgroup analysis of the Japanese patient subset from TEMPO 3:4 trial.Clinical and experimental nephrology · 2015Trial
- Nationwide attribute-based cross-classification reveals distinct risk patterns of major complications in ADPKD.Clinical and experimental nephrology · 2026Article
- Association of anemia with long-term renal prognosis in autosomal dominant polycystic kidney disease using time-series analysis.Scientific reports · 2026Article
- Machine learning for predicting CKD stages in patients with autosomal dominant polycystic kidney disease: a nationwide cohort study in Japan.Scientific reports · 2026Article
- Association of hyperphosphatemia with renal prognosis in patients with autosomal dominant polycystic kidney disease.Clinical and experimental nephrology · 2025Article
- Elevated hemoglobin levels in renal transplant recipients with polycystic kidney disease versus other etiologies: exploring mechanisms and implications for outcomes.Journal of nephrology · 2024Article
- Factors associated with early-onset intracranial aneurysms in patients with autosomal dominant polycystic kidney disease.Journal of nephrology · 2024Article
- Public support for patients with intractable diseases in Japan: impact on clinical indicators from nationwide registries in patients with autosomal dominant polycystic kidney disease.Clinical and experimental nephrology · 2023Article
- Case Report: Guanfacine and methylphenidate improved chronic lower back pain in autosomal dominant polycystic kidney disease with comorbid attention deficit hyperactivity disorder and autism spectrum disorder.Frontiers in pediatrics · 2023Article
- Impact of kidney function and kidney volume on intracranial aneurysms in patients with autosomal dominant polycystic kidney disease.Scientific reports · 2022Article
- Mutation Type and Intracranial Aneurysm Formation in Autosomal Dominant Polycystic Kidney Disease.Stroke (Hoboken, N.J.) · 2022Article
- Germline Mutations for Kidney Volume in ADPKD.Kidney international reports · 2022Article
- Aggressive advanced gastric cancer in a patient with autosomal dominant polycystic kidney disease.Clinical journal of gastroenterology · 2021Article
- Predicting liver cyst severity by mutations in patients with autosomal-dominant polycystic kidney disease.Hepatology international · 2021Article
- Article
- A Multitubular Kidney-on-Chip to Decipher Pathophysiological Mechanisms in Renal Cystic Diseases.Frontiers in bioengineering and biotechnology · 2021Article
- Novel Potential Application of Chitosan Oligosaccharide for Attenuation of Renal Cyst Growth in the Treatment of Polycystic Kidney Disease.Molecules (Basel, Switzerland) · 2020Article
- Laparoscopic ureterolithotomy, flexible ureteroscopic lithotripsy and percutaneous nephrolithotomy for treatment of upper urinary calculi in patients with autosomal dominant polycystic kidney disease.Clinical and experimental nephrology · 2020Article
- Association between anemia and renal prognosis in autosomal dominant polycystic kidney disease: a retrospective study.Clinical and experimental nephrology · 2020Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is the most common progressive hereditary kidney disease. In 85-90% of cases, ADPKD results from a mutation in the PKD1 gene, and the other 10-15% of the cases are accounted for by mutations in PKD2. PKD1 and PKD2 encode polycystin-1 and polycystin-2. Polycystin-1 may be a receptor that controls the channel activity of polycystin-2 as part of the polycystin signaling complex. ADPKD is characterized by the progressive development of fluid-filled cysts derived from renal tubular epithelial cells that gradually compress the parenchyma and compromise renal function. In recent years, considerable interest has developed in the primary cilia as a site of the proteins that are involved in renal cystogenesis. The pathological processes that facilitate cyst enlargement are hypothesized to result from two specific cellular abnormalities: (1) increased fluid secretion into the cyst lumen and (2) inappropriately increased cell division by the epithelium lining the cyst. Since there is no clinically approved specific or targeted therapy, current practice focuses on blood pressure control and statin therapy to reduce the cardiac mortality associated with chronic kidney disease. However, recent advances in our understanding of the pathways that govern renal cystogenesis have led to a number of intriguing possibilities in regard to therapeutic interventions. The purpose of this article is to review the pathogenesis of renal cyst formation and to review novel targets for the treatment of ADPKD.
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