Establishment of a novel human CIC-DUX₄ sarcoma cell line, Kitra-SRS, with autocrine IGF-1R activation and metastatic potential to the lungs.

ABSTRACT: Approximately 60-70% of EWSR1-negative small blue round cell sarcomas harbour a rearrangement of CIC, most commonly CIC-DUX₄. CIC-DUX₄ sarcoma (CDS) is an aggressive and often fatal high-grade sarcoma appearing predominantly in children and young adults. Although cell lines and their xenograft models are essential tools for basic research and development of antitumour drugs, few cell lines currently exist for CDS. We successfully established a novel human CDS cell line designated Kitra-SRS and developed orthotopic tumour xenografts in nude mice. The CIC-DUX₄ fusion gene in Kitra-SRS cells was generated by t(12;19) complex chromosomal rearrangements with an insertion of a chromosome segment including a DUX₄ pseudogene component. Kitra-SRS xenograf

SUBMITTER: Nakai S 

PROVIDER: S-EPMC6825133 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature