Molecular cytogenetic characterization of two established ESFT cell lines
Molecular cytogenetic characterization of two established ESFT cell lines
复制标题
DOI：
10.1007/s13577-016-0145-7
复制
发表时间：
2016
期刊：
影响因子：
4.3
通讯作者：
M. Ishiguro;Mutsumi Yuki;T. Fukushige;M. Mizoguchi;Y. Kaneko;Takeshita Morishige;H. Iwasaki
中科院分区：
文献类型：
--
作者：
M. Ishiguro;Mutsumi Yuki;T. Fukushige;M. Mizoguchi;Y. Kaneko;Takeshita Morishige;H. Iwasaki
尤文肉瘤/原始神经外胚层肿瘤/阿斯金瘤(Ewing‘s Saroma Family of Tuments：ESFT)是儿童和青壮年最常见的骨和软组织恶性肿瘤，形态上属于一组小圆细胞肿瘤。在此，我们建立了两个人ESFT细胞系，FU-PNET-3和FU-PNET-4，分别来自髂骨和胸壁，这两个细胞系的细胞在免疫缺陷小鼠中都有致瘤作用。组织学上，原发和异种移植瘤及培养细胞均由CD99和Nkx2.2免疫阳性的小圆形细胞组成。分子生物学检测表明，在FU-PNET-3细胞中存在EWSR1外显子7至FLI1外显子6的嵌合转录，在FU-PNET-4细胞中存在EWSR1外显子10至FLI1外显子6的嵌合转录。细胞遗传学分析显示t(11；22)(q24；q12)易位，两种培养细胞均有继发性改变。这些组织学、分子生物学和细胞遗传学的发现表明，这两种细胞系都存在ESFT。ESFT已被广泛研究，但其反复出现的融合基因具有异质性，其生物学行为尚不清楚。FU-PNET-3和FU-PNET-4细胞系已经被很好地检测，并可能成为研究ESFT的遗传和生物学行为特性的有用工具。
Ewing’s sarcoma/primitive neuroectodermal tumor/Askin’s tumor (Ewing`s sarcoma family of tumors: ESFT) is the most common type of malignant tumor of bone and soft tissue in children and young adults, and morphologically is a member of a group of small round cell tumors. We report, here, on the establishment of two human ESFT cell lines, FU-PNET-3 and FU-PNET-4, from the iliac and the chest wall, respectively, the cells of both cell lines were tumorigenic in immunodeficient mice. Histologically, both original and xenograft tumors and cultured cells were composed of small round cells with positive immunoreactivity for CD99 and Nkx2.2. Molecular biological examination demonstrated chimeric transcripts ofEWSR1exon 7 toFLI1exon 6 in FU-PNET-3 cells, andEWSR1exon 10 toFLI1exon 6 in FU-PNET-4 cells. Cytogenetic analysis revealed chromosome translocation t(11;22)(q24;q12) and some secondary changes in both cultured cells. These histological, molecular biological, and cytogenetical findings indicate ESFT in both cell lines. ESFT is well studied, but its recurrent fusion genes are heterogeneous and its biological behaviors are unclear. The FU-PNET-3 and FU-PNET-4 cell lines have been well examined and may become useful tools for studying the genetic and biological behavioral properties of ESFT.