ArticleDisease models & mechanisms2016
Loss of vhl in the zebrafish pronephros recapitulates early stages of human clear cell renal cell carcinoma.
Article in Disease models & mechanisms, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 28 citations in OpenAlex.
- Article
- Discovery of tumor antigens in renal cell carcinoma and prospects for clinical application.Frontiers in immunology · 2026Review
- APOC2 Promotes Clear Cell Renal Cell Carcinoma Progression via Activation of the JAK-STAT Signaling Pathway.Current issues in molecular biology · 2025Article
- Zebrafish-A Suitable Model for Rapid Translation of Effective Therapies for Pediatric Cancers.Cancers · 2024Review
- A novel lipid metabolism gene signature for clear cell renal cell carcinoma using integrated bioinformatics analysis.Frontiers in cell and developmental biology · 2023Article
- Expression and Variations inGenes · 2022Article
- Establishment of a ccRCC patient-derived chick chorioallantoic membrane model for drug testing.Frontiers in medicine · 2022Article
- The Zebrafish Model to Understand Epigenetics in Renal Diseases.International journal of molecular sciences · 2021Review
- TET is targeted for proteasomal degradation by the PHD-pVHL pathway to reduce DNA hydroxymethylation.The Journal of biological chemistry · 2020Article
- Tumor-derived exosomes facilitate tumor cells escape from drug therapy in clear cell renal cell carcinoma.Translational cancer research · 2020Article
- Ciliary Genes in Renal Cystic Diseases.Cells · 2020Review
- Choosing The Right Animal Model for Renal Cancer Research.Translational oncology · 2020Review
- Article
- Generation of autochthonous mouse models of clear cell renal cell carcinoma: mouse models of renal cell carcinoma.Experimental & molecular medicine · 2018Review
- The von Hippel-Lindau Gene Is Required to Maintain Renal Proximal Tubule and Glomerulus Integrity in Zebrafish Larvae.Nephron · 2018Article
- Zebrafish as a Model for Drug Screening in Genetic Kidney Diseases.Frontiers in pediatrics · 2018Review
- Renal Cell Carcinoma in von Hippel-Lindau Disease-From Tumor Genetics to Novel Therapeutic Strategies.Frontiers in pediatrics · 2018Review
- Role of primary cilia in non-dividing and post-mitotic cells.Cell and tissue research · 2017Review
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 2 countries.
Funding
Abstract
Patients with von Hippel-Lindau (VHL) disease harbor a germline mutation in the VHL gene leading to the development of several tumor types including clear cell renal cell carcinoma (ccRCC). In addition, the VHL gene is inactivated in over 90% of sporadic ccRCC cases. 'Clear cell' tumors contain large, proliferating cells with 'clear cytoplasm', and a reduced number of cilia. VHL inactivation leads to the stabilization of hypoxia inducible factors 1a and 2a [HIF1a and HIF2a (HIF2a is also known as EPAS1)] with consequent up-regulation of specific target genes involved in cell proliferation, angiogenesis and erythropoiesis. A zebrafish model with a homozygous inactivation in the VHL gene (vhl(-/-)) recapitulates several aspects of the human disease, including development of highly vascular lesions in the brain and the retina and erythrocytosis. Here, we characterize for the first time the epithelial abnormalities present in the kidney of the vhl(-/-) zebrafish larvae as a first step in building a model of ccRCC in zebrafish. Our data show that the vhl(-/-) zebrafish kidney is characterized by an increased tubule diameter, disorganized cilia, the dramatic formation of cytoplasmic lipid vesicles, glycogen accumulation, aberrant cell proliferation and abnormal apoptosis. This phenotype of the vhl(-/-) pronephros is reminiscent of clear cell histology, indicating that the vhl(-/-) mutant zebrafish might serve as a model of early stage RCC. Treatment of vhl(-/-) zebrafish embryos with a small-molecule HIF2a inhibitor rescued the pronephric abnormalities, underscoring the value of the zebrafish model in drug discovery for treatment of VHL disease and ccRCC.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.