Guha A, Glowacka D, Carroll R, Dashner K, Black P M, Stiles C D
Dana-Farber Cancer Institute, Boston, USA.
J Neurol Neurosurg Psychiatry. 1995 Jun;58(6):711-4. doi: 10.1136/jnnp.58.6.711.
Expression of platelet derived growth factor (PDGF) and PDGF-receptor mRNA was examined from a glioblastoma taken from a patient with Li-Fraumeni syndrome. Northern blot analysis and in situ hybridisation showed very high concentrations of both PDGF-A and PDGF alpha-receptor mRNA in the tumour. The overall pattern of PDGF expression was similar to those found in sporadic glioblastomas. Mutations in p53 has been implicated as an early pathogenic event leading to sporadic low grade astrocytomas, and is the third most common tumour type in patients with Li-Fraumeni syndrome, where they are predisposed due to a germline mutation in the p53 tumour suppressor gene. This study suggests that progression towards a glioblastoma in both the general population and in patients with Li-Fraumeni syndrome may involve potential autocrine and paracrine stimulation by growth factors such as PDGF.
从一名患有李-弗劳梅尼综合征的患者的胶质母细胞瘤中检测血小板衍生生长因子(PDGF)和PDGF受体mRNA的表达。Northern印迹分析和原位杂交显示肿瘤中PDGF-A和PDGFα受体mRNA的浓度都非常高。PDGF表达的总体模式与散发性胶质母细胞瘤中的相似。p53突变被认为是导致散发性低级别星形细胞瘤的早期致病事件,并且是李-弗劳梅尼综合征患者中第三常见的肿瘤类型,在这些患者中,由于p53肿瘤抑制基因的种系突变而易患肿瘤。这项研究表明,在普通人群和李-弗劳梅尼综合征患者中,向胶质母细胞瘤的进展可能涉及生长因子如PDGF的潜在自分泌和旁分泌刺激。