ArticleAmerican journal of physiology. Renal physiology2014
Evidence for pericyte origin of TSC-associated renal angiomyolipomas and implications for angiotensin receptor inhibition therapy.
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 26 papers.
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Who cites it
26 citing papers in PubMed, 51 citations in OpenAlex.
- Effect of everolimus on renal function in patients with tuberous sclerosis complex: evidence from EXIST-1 and EXIST-2.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2019Trial
- Everolimus long-term use in patients with tuberous sclerosis complex: Four-year update of the EXIST-2 study.PloS one · 2017Trial
- Pharmacokinetics and pharmacodynamics of everolimus in patients with renal angiomyolipoma and tuberous sclerosis complex or lymphangioleiomyomatosis.British journal of clinical pharmacology · 2016Trial
- Correlations between TSC Expression and Menorrhagia in Adenomyosis Patients.Reproductive sciences (Thousand Oaks, Calif.) · 2026Article
- Angiotensin II drives proliferation and extracellular matrix deposition in human uterine fibroid cells in vitro.F&S science · 2026Article
- Spatial gene expression profiling reveals the distinct microenvironment of tuberous sclerosis complex-associated angiomyolipoma.Scientific reports · 2025Article
- SFPQ-TFE3 reciprocally regulates mTORC1 and induces lineage plasticity in a mouse model of renal tumorigenesis.Nature communications · 2025Article
- Insights into Tuberous Sclerosis Complex : From Genes to Clinics.Journal of Korean Neurosurgical Society · 2025Article
- Midkine expression by stem-like tumor cells drives persistence to mTOR inhibition and an immune-suppressive microenvironment.Nature communications · 2022Article
- GPNMB expression identifies TSC1/2/mTOR-associated and MiT family translocation-driven renal neoplasms.The Journal of pathology · 2022Article
- Renal cystic disease in tuberous sclerosis complex.Experimental biology and medicine (Maywood, N.J.) · 2021Review
- The effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures.Scientific reports · 2021Article
- Sporadic, classic-type renal angiomyolipoma with renal vein and inferior vena cava extension: an incidental case.BMJ case reports · 2021Article
- Tuberous sclerosis complex exhibits a new renal cystogenic mechanism.Physiological reports · 2019Article
- A novel mouse model of hemangiopericytoma due to loss of Tsc2.Human molecular genetics · 2018Article
- Renal disease in tuberous sclerosis complex: pathogenesis and therapy.Nature reviews. Nephrology · 2018Review
- Renal progression factors in young patients with tuberous sclerosis complex: a retrospective cohort study.Pediatric nephrology (Berlin, Germany) · 2018Article
- Evidence of renal angiomyolipoma neoplastic stem cells arising from renal epithelial cells.Nature communications · 2017Article
- Peroxisome proliferator-activated receptor gamma (PPARγ) is central to the initiation and propagation of human angiomyolipoma, suggesting its potential as a therapeutic targetEMBO molecular medicine · 2017Article
- Combination of Everolimus with Sorafenib for Solid Renal Tumors in Tsc2Neoplasia (New York, N.Y.) · 2017Article
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Authors and funding
12 authors at 7 institutions in 2 countries.
Funding
Abstract
Nearly all patients with tuberous sclerosis complex (TSC) develop renal angiomyolipomas, although the tumor cell of origin is unknown. We observed decreased renal angiomyolipoma development in patients with TSC2- polycystic kidney disease 1 deletion syndrome and hypertension that were treated from an early age with angiotensin-converting enzyme inhibitors or angiotensin receptor blockers compared with patients who did not receive this therapy. TSC-associated renal angiomyolipomas expressed ANG II type 1 receptors, platelet-derived growth factor receptor-β, desmin, α-smooth muscle actin, and VEGF receptor 2 but did not express the adipocyte marker S100 or the endothelial marker CD31. Sera of TSC patients exhibited increased vascular mural cell-secreted peptides, such as VEGF-A, VEGF-D, soluble VEGF receptor 2, and collagen type IV. These findings suggest that angiomyolipomas may arise from renal pericytes. ANG II treatment of angiomyolipoma cells in vitro resulted in an exaggerated intracellular Ca(2+) response and increased proliferation, which were blocked by the ANG II type 2 receptor antagonist valsartan. Blockade of ANG II signaling may have preventative therapeutic potential for angiomyolipomas.
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