ArticleOncotarget2016
Aberrant epigenetic regulation in clear cell sarcoma of the kidney featuring distinct DNA hypermethylation and EZH2 overexpression.
Article in Oncotarget, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 26 citations in OpenAlex.
- Relapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration.Nature reviews. Urology · 2026Review
- NCBP1 promotes the malignant progression of Wilms' tumor through stabilization of KPNA2 mRNA.Cancer gene therapy · 2026Article
- Review
- Wilms' Tumor: A Review of Clinical Characteristics, Treatment Advances, and Research Opportunities.Medicina (Kaunas, Lithuania) · 2025Review
- Comprehensive prognostic gene identification and functional characterization of GRAMD1A in Wilms tumor: development of risk prediction models and therapeutic implications.Frontiers in oncology · 2024Article
- Molecular Signature of Biological Aggressiveness in Clear Cell Sarcoma of the Kidney (CCSK).International journal of molecular sciences · 2023Article
- Identification of key genes and validation of key gene aquaporin 1 on Wilms' tumor metastasis.PeerJ · 2023Article
- Paediatric BCOR-associated sarcomas with a novel long spliced internal tandem duplication of BCOR exon 15.The journal of pathology. Clinical research · 2022Article
- Bioinformatics analysis and identification of genes and pathways involved in patients with Wilms tumor.Translational cancer research · 2022Article
- Identification of a 12-Gene Signature and Hub Genes Involved in Kidney Wilms TumorFrontiers in oncology · 2022Article
- Article
- Cancer Stem Cells in Soft-Tissue Sarcomas.Cells · 2020Review
- Clear cell sarcoma of the kidney in children: a clinopathologic analysis of three cases.International journal of clinical and experimental pathology · 2020Article
- Aberrant Epigenetic Regulation in Head and Neck Cancer Due to Distinct EZH2 Overexpression and DNA Hypermethylation.International journal of molecular sciences · 2018Article
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Authors and funding
6 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The global methylation profile and the mutational status of 633 specific epigenetic regulators were analyzed in the pediatric tumor clear cell sarcoma of the kidney (CCSK). Methylation array analyses of 30 CCSKs revealed CCSK tumor DNA to be globally hypermethylated compared to Wilms tumor, normal fetal kidney, and adult kidney. The aberrant methylation pattern of CCSKs was associated with activation of genes involved in embryonic processes and with silencing of genes linked to normal kidney function. No epigenetic regulator was recurrently mutated in our cohort, but a mutation in the key epigenetic regulator EZH2 was discovered in one case. EZH2 mRNA was significantly higher in CCSK compared to Wilms tumor and normal kidney, and the EZH2 protein was strongly expressed in more than 90 % of CCSK tumor cells in 9/9 tumors analyzed. This was in striking contrast to the lack of EZH2 protein expression in Wilms tumor stromal elements, indicating that EZH2 could be explored further as a diagnostic marker and a potential drug target for CCSK.
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