骨外黏液样软骨肉瘤中受EWS/NR4A3融合蛋白调控基因的鉴定
Identification of genes regulated by the EWS/NR4A3 fusion protein in extraskeletal myxoid chondrosarcoma.
作者信息
Filion Christine, Labelle Yves
机构信息
Centre de recherche de l'Hôpital Saint-François d'Assise/CHUQ, 10 rue de l'Espinay, local D0-711, Québec, QC, Canada.
出版信息
Tumour Biol. 2012 Oct;33(5):1599-605. doi: 10.1007/s13277-012-0415-2. Epub 2012 May 18.
Approximately 75 % of extraskeletal myxoid chondrosarcoma tumors (EMC) harbor a t(9;22) chromosome translocation generating an EWS/NR4A3 fusion protein that is thought to be instrumental in the tumoral process. Current evidence suggests that one function of the fusion protein is to overexpress target genes. We have generated an in vitro human cellular model in which the fusion protein is expressed in mesenchymal bone marrow stem cells. We have performed microarray analyses of these cells and identified several genes overexpressed in the presence of EWS/NR4A3 which are also overexpressed in EMC tumors. These genes and their products represent potential therapeutic targets for EMC tumors.
约75%的骨外黏液样软骨肉瘤（EMC）肿瘤存在t(9;22)染色体易位，产生EWS/NR4A3融合蛋白，该蛋白被认为在肿瘤发生过程中起重要作用。目前的证据表明，融合蛋白的一个功能是使靶基因过表达。我们构建了一个体外人类细胞模型，其中融合蛋白在间充质骨髓干细胞中表达。我们对这些细胞进行了微阵列分析，并鉴定出几个在EWS/NR4A3存在时过表达的基因，这些基因在EMC肿瘤中也过表达。这些基因及其产物代表了EMC肿瘤潜在的治疗靶点。
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