ArticlePloS one2013
A novel chordoma xenograft allows in vivo drug testing and reveals the importance of NF-κB signaling in chordoma biology.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
13 citing papers in PubMed, 25 citations in OpenAlex.
- Analysis of long-term TNF-alpha induced EGFR tyrosine kinase inhibitor resistance in chordoma.RSC medicinal chemistry · 2025Article
- SMARCB1 orchestrates cellular plasticity and oncogenic pathways in typical and chondroid chordomas.Scientific reports · 2025Article
- Virus-like particle-based delivery of Cas9/guide RNA ribonucleoprotein efficiently edits the brachyury gene and inhibits chordoma growth in vivo.Discover oncology · 2023Article
- High-Throughput/High Content Imaging Screen Identifies Novel Small Molecule Inhibitors and Immunoproteasomes as Therapeutic Targets for Chordoma.Pharmaceutics · 2023Article
- Article
- Personalized chordoma organoids for drug discovery studies.Science advances · 2022Article
- Selinexor inhibits growth of patient derived chordomasFrontiers in oncology · 2022Article
- A chronicle review of new techniques that facilitate the understanding and development of optimal individualized therapeutic strategies for chordoma.Frontiers in oncology · 2022Review
- The role of TNF-α in chordoma progression and inflammatory pathways.Cellular oncology (Dordrecht, Netherlands) · 2019Article
- UM-Chor1: establishment and characterization of the first validated clival chordoma cell line.Journal of neurosurgery · 2018Article
- Chordoma: an update on the pathophysiology and molecular mechanisms.Current reviews in musculoskeletal medicine · 2015Article
- Article
- PD-1, PD-L1, PD-L2 expression in the chordoma microenvironment.Journal of neuro-oncology · 2015Article
Corrections and comments
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Authors and funding
16 authors at 7 institutions in 1 country.
Funding
Abstract
Chordoma is a rare primary bone malignancy that arises in the skull base, spine and sacrum and originates from remnants of the notochord. These tumors are typically resistant to conventional chemotherapy, and to date there are no FDA-approved agents to treat chordoma. The lack of in vivo models of chordoma has impeded the development of new therapies for this tumor. Primary tumor from a sacral chordoma was xenografted into NOD/SCID/IL-2R γ-null mice. The xenograft is serially transplantable and was characterized by both gene expression analysis and whole genome SNP genotyping. The NIH Chemical Genomics Center performed high-throughput screening of 2,816 compounds using two established chordoma cell lines, U-CH1 and U-CH2B. The screen yielded several compounds that showed activity and two, sunitinib and bortezomib, were tested in the xenograft. Both agents slowed the growth of the xenograft tumor. Sensitivity to an inhibitor of IκB, as well as inhibition of an NF-κB gene expression signature demonstrated the importance of NF-κB signaling for chordoma growth. This serially transplantable chordoma xenograft is thus a practical model to study chordomas and perform in vivo preclinical drug testing.
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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.