Telethon Institute for Child Health Research and Centre for Child Health Research, University of Western Australia, Perth, Australia.
Neuropathology. 2009 Aug;29(4):398-409. doi: 10.1111/j.1440-1789.2008.00989.x. Epub 2008 Dec 11.
Medulloblastoma (MB) is the most common type of brain tumor affecting children. These tumors are a significant cause of childhood mortality and morbidity, and more effective and less invasive treatment options are urgently required. To achieve these aims, it will be critical to develop a more comprehensive understanding of the molecular pathogenesis of MB. At present, there are relatively few well-characterized MB cell lines available to the research community for the study of MB molecular and cellular biology. Here we present the case reports of two children diagnosed with classic and desmoplastic MB, and describe the characteristics of two new MB cell lines derived from these individuals. A number of genes encoding components of the sonic hedgehog (SHH) and WNT pathways were up-regulated in the desmoplastic relative to the classic MB cell line consistent with aberrant activation of these pathways in desmoplastic MB. These cell lines represent an additional resource for the analysis of diverse aspects of MB biology.
髓母细胞瘤(MB)是影响儿童的最常见脑肿瘤类型。这些肿瘤是儿童死亡和发病的重要原因,迫切需要更有效和侵入性更小的治疗选择。为了实现这些目标,必须更全面地了解 MB 的分子发病机制。目前,可供研究界研究 MB 分子和细胞生物学的经过良好表征的 MB 细胞系相对较少。在这里,我们报告了两名被诊断患有经典型和促结缔组织增生型 MB 的儿童的病例报告,并描述了源自这两个人的两种新的 MB 细胞系的特征。与经典型 MB 细胞系相比,促结缔组织增生型 MB 细胞系中编码 sonic hedgehog(SHH)和 WNT 途径组成部分的基因上调,表明促结缔组织增生型 MB 中这些途径的异常激活。这些细胞系代表了分析 MB 生物学各个方面的另一个资源。