Gangliosides in neuroblastomas.

C L Schengrund, S J Shochat
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引用次数: 24

Abstract

Neuroblastomas from children presenting with tumors at various ages and different primary sites (abdominal, adrenals, pelvic, and thoracic) were studied. Analysis of the ganglioside patterns of 53 tumors indicated that patients who were either disease positive 2 yr following surgery or dead of disease, had significantly (p less than 0.005) less GT1b plus GD1b than tumors from patients that were disease free 2 yr post surgery. The presence of GD2 in 45 of the tumors correlates well with the suggestion that it can be used as a marker in neuroblastoma diagnosis. Children with thoracic neuroblastomas have a significantly better prognosis than children with tumors in other anatomic sites. Analysis of the ganglioside composition of these tumors only, indicated that they had a significantly higher (p less than 0.005) concentration of GT1b and GD1b and a significantly lower concentration (p less than 0.025) of monosialogangliosides than those patients who were dead of disease or had persistent disease. These results suggest that low levels of GT1b and GD1b correlate with a poor prognosis. The thoracic neuroblastomas may be comprised of more "differentiated" neuroblastoma cells (ganglioside patterns more similar to the CNS), and this may contribute to the fact that about 85% of children with thoracic neuroblastoma recover. To understand why the ganglioside pattern may serve as a prognostic indicator for neuroblastoma, it is necessary to know whether gangliosides have specific roles in neuronal differentiation. Our approach to this question is to compare the effect(s) of added ganglioside or the corresponding oligosaccharide on neuroblastoma cells. Results obtained suggest that the oligosaccharide from GM1 is able to enhance neuritogenesis by S20Y murine neuroblastoma cells to the same extent that GM1 does.

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神经母细胞瘤中的神经节苷类。
我们研究了来自不同年龄和不同原发部位(腹部、肾上腺、骨盆和胸部)的儿童神经母细胞瘤。对53例肿瘤的神经节苷类分析表明,术后2年疾病阳性或疾病死亡患者的GT1b + GD1b明显低于术后2年无疾病患者的肿瘤(p < 0.005)。GD2在45个肿瘤中的存在与它可以作为神经母细胞瘤诊断的标记物的建议密切相关。胸部神经母细胞瘤患儿预后明显优于其他解剖部位肿瘤患儿。仅对这些肿瘤的神经节苷脂组成进行分析表明,与死于疾病或有持续性疾病的患者相比,这些肿瘤的GT1b和GD1b浓度显著高于(p < 0.005),单口神经节苷脂浓度显著低于(p < 0.025)。这些结果表明,低水平的GT1b和GD1b与预后不良相关。胸椎神经母细胞瘤可能由更“分化”的神经母细胞瘤细胞组成(神经节苷脂模式更类似于中枢神经系统),这可能是约85%胸椎神经母细胞瘤患儿康复的原因。为了理解为什么神经节苷脂模式可以作为神经母细胞瘤的预后指标,有必要了解神经节苷脂是否在神经元分化中具有特定的作用。我们解决这个问题的方法是比较添加神经节苷脂或相应的低聚糖对神经母细胞瘤细胞的影响。结果表明,GM1寡糖对S20Y小鼠神经母细胞瘤细胞的神经发生促进作用与GM1相同。
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