Characterization of 2 Novel Ependymoma Cell Lines With Chromosome 1q Gain Derived From Posterior Fossa Tumors of Childhood
| Autor: | Rajeev Vibhakar, Eric Prince, Kathleen Dorris, Nicholas K. Foreman, Seth Lummus, Lindsey M. Hoffman, Vladimir Amani, Todd C. Hankinson, Andrea Griesinger, Andrew M. Donson, Michael H. Handler, Davis Witt |
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| Rok vydání: | 2017 |
| Předmět: |
Male
0301 basic medicine Ependymoma Pathology medicine.medical_specialty Derivative chromosome medicine.medical_treatment Brain tumor Infratentorial Neoplasms Biology Pathology and Forensic Medicine Transcriptome 03 medical and health sciences Cellular and Molecular Neuroscience Imaging Three-Dimensional Receptors HIV 0302 clinical medicine In vivo Cell Line Tumor Glial Fibrillary Acidic Protein medicine Humans Child Chromosome Aberrations Microscopy Confocal Gene Expression Profiling Mucin-1 Nuclear Proteins Chromosome Original Articles General Medicine Microarray Analysis medicine.disease DNA-Binding Proteins Radiation therapy Ki-67 Antigen 030104 developmental biology Neurology Chromosomes Human Pair 1 Cell culture 030220 oncology & carcinogenesis Cytogenetic Analysis Neurology (clinical) Transcription Factors |
| Zdroj: | Journal of Neuropathology & Experimental Neurology. 76:595-604 |
| ISSN: | 1554-6578 0022-3069 |
| DOI: | 10.1093/jnen/nlx040 |
| Popis: | Ependymoma (EPN) is a common brain tumor of childhood that, despite standard surgery and radiation therapy, has a relapse rate of 50%. Clinical trials have been unsuccessful in improving outcome by addition of chemotherapy, and identification of novel therapeutics has been hampered by a lack of in vitro and in vivo models. We describe 2 unique EPN cell lines (811 and 928) derived from recurrent intracranial metastases. Both cell lines harbor the high-risk chromosome 1q gain (1q+) and a derivative chromosome 6, and both are classified as molecular group A according to transcriptomic analysis. Transcriptional enrichment of extracellular matrix-related genes was a common signature of corresponding primary tumors and cell lines in both monolayer and 3D formats. EPN cell lines, when cultured in 3D format, clustered closer to the primary tumors with better fidelity of EPN-specific transcripts than when grown as a monolayer. Additionally, 3D culture revealed ependymal rosette formation and cilia-related ontologies, similar to in situ tumors. Our data confirm the validity of the 811 and 928 cell lines as representative models of intracranial, posterior fossa 1q+ EPN, which holds potential to advance translational science for patients affected by this tumor. |
| Databáze: | OpenAIRE |
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