| Abstract |
Ependymoma, a tumor of the ependymal cells that line the walls of the ventricles, is a rare disease with a poor prognosis. There is a need for academia-led efforts to understand the mechanisms of ependymoma formation and to explore new therapeutic targets, because ependymomas are resistant to chemotherapy and difficult to treat surgically. The most aggressive subclass of supratentorial ependymomas have been reported to express the fusion protein ZFTA-RELA(named C11orf95-RELA at the time of application). In the present study, phenotypic analysis of ZFTA-deficient mice was performed with the aim of investigating whether serious side effects could appear in ependymoma treatment strategies based on functional inhibition of ZFTA-RELA. We found that ZFTA-deficient mice develop and grow normally and that functional inhibition of ZFTA-RELA is a promising ependymoma treatment strategy. From fungal extracts, we identified epi-Aszonalenin B as a compound that inhibits the NF-κB oncogenic signaling pathway that is activated by the expression of ZFTA-RELA. epi-Aszonalenin B is expected to be a lead compound for the development of ependymoma therapeutics.
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