ArticleNeuro-oncology2010
A clinically relevant orthotopic xenograft model of ependymoma that maintains the genomic signature of the primary tumor and preserves cancer stem cells in vivo.
Article in Neuro-oncology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 77 papers.
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Who cites it
77 citing papers in PubMed, 100 citations in OpenAlex.
- A patient-derived organoid platform for predominantly posterior fossa adult ependymoma: histopathologic preservation, culture-associated transcriptomic remodeling, and in vivo tumorigenicity.Journal of neuro-oncology · 2026Article
- Sirolimus potentiates oncolytic Virus M1 efficacy through tumor-selective augmentation of viral replication.Acta pharmacologica Sinica · 2026Article
- A tumor-associated photoreceptor signature unifies distinct central nervous system malignancies.Cancer cell · 2026Article
- RASA2 deletion rescues immune synapse dysfunction, enhancing CAR T cell efficacy against DMGs.Journal for immunotherapy of cancer · 2026Article
- Targeting Pediatric Glioblastomas by Combining OLIG2 Inhibitor CT-179 with Fractionated Radiation in a Panel of Patient-Derived Orthotopic Xenograft Mouse Models.International journal of molecular sciences · 2026Article
- Modeling pediatric brain tumors with human stem cells.Frontiers in cellular neuroscience · 2026Review
- TRP Channel Inhibition and NF-κB Pathway Suppression in Human Ependymal Tumor cell-line by Achillea Biebersteinii Aqueous Extract.Cell biochemistry and biophysics · 2025Article
- Profiling extracellular vesicles from cerebrospinal fluid for classification of intradural spinal tumors.Scientific reports · 2025Article
- B7-H3 CAR T Cells Are Effective against Ependymomas but Limited by Tumor Size and Immune Response.Clinical cancer research : an official journal of the American Association for Cancer Research · 2025Article
- Current Landscape of Preclinical Models for Pediatric Gliomas: Clinical Implications and Future Directions.Cancers · 2025Review
- Targeting EPHB2/ABL1 restores antitumor immunity in preclinical models of ependymoma.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- CD57 defines a novel cancer stem cell that drive invasion of diffuse pediatric-type high grade gliomas.British journal of cancer · 2024Article
- Article
- Patient-Derived Models of Cancer in the NCI PDMC Consortium: Selection, Pitfalls, and Practical Recommendations.Cancers · 2024Review
- Review
- RFX2 promotes tumor cell stemness through epigenetic regulation of PAF1 in spinal ependymoma.Journal of neuro-oncology · 2023Article
- GRP78-CAR T cell effector function against solid and brain tumors is controlled by GRP78 expression on T cells.Cell reports. Medicine · 2023Article
- The development of a rapid patient-derived xenograft model to predict chemotherapeutic drug sensitivity/resistance in malignant glial tumors.Neuro-oncology · 2023Article
- Human Patient-Derived Brain Tumor Models to Recapitulate Ependymoma Tumor Vasculature.Bioengineering (Basel, Switzerland) · 2023Article
- Targeting GBM with an Oncolytic Picornavirus SVV-001 alone and in combination with fractionated Radiation in a Novel Panel of Orthotopic PDX models.Journal of translational medicine · 2023Article
17 more citing papers are in PubMed but not listed here.
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Authors and funding
19 authors at 1 institution in 1 country.
Funding
Abstract
Limited availability of in vitro and in vivo model systems has hampered efforts to understand tumor biology and test novel therapies for ependymoma, the third most common malignant brain tumor that occurs in children. To develop clinically relevant animal models of ependymoma, we directly injected a fresh surgical specimen from a 9-year-old patient into the right cerebrum of RAG2/severe complex immune deficiency (SCID) mice. All five mice receiving the initial transplantation of the patient tumor developed intracerebral xenografts, which have since been serially subtransplanted in vivo in mouse brains for 4 generations and can be cryopreserved for long-term maintenance of tumorigenicity. The xenograft tumors shared nearly identical histopathological features with the original tumors, harbored 8 structural chromosomal abnormalities as detected with spectral karyotyping, maintained gene expression profiles resembling that of the original patient tumor with the preservation of multiple key genetic abnormalities commonly found in human ependymomas, and contained a small population (<2.2%) of CD133(+) stem cells that can form neurospheres and display multipotent capabilities in vitro. The permanent cell line (BXD-1425EPN), which was derived from a passage II xenograft tumor and has been passaged in vitro more than 70 times, expressed similar differentiation markers of the xenograft tumors, maintained identical chromosomal abnormalities, and formed tumors in the brains of SCID mice. In conclusion, direct injection of primary ependymoma tumor cells played an important role in the generation of a clinically relevant mouse model IC-1425EPN and a novel cell line, BXD-1425EPN. This cell line and model will facilitate the biological studies and preclinical drug screenings for pediatric ependymomas.
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Registered trials
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.