Gustafsson B, Stål O, Gustafsson B
Department of Paediatrics, Huddinge Hospital, Karolinska Institute, Huddinge, Sweden.
Pediatr Hematol Oncol. 1998 Nov-Dec;15(6):519-26. doi: 10.3109/08880019809018313.
Mutation of the TP53 gene is one of the most common molecular alterations in a variety of tumors, but it occurs infrequently in childhood and adult hematological malignancies. Protein accumulation often results from mutations that lead to inactivation of the p53 protein. Other causes of functional inactivation of the p53 protein include stabilization of p53 via proteins such as MDM2, an oncoprotein capable of forming specific complexes with p53. In the present study, protein expressions of MDM2 and p53 were investigated by immunohistochemistry from bone marrow samples in 23 patients with acute lymphoblastic leukemia aged 1-13 years at diagnosis. p53 protein overexpression was detected in only one case, while overexpression of MDM2 was detected in samples from five patients. All five patients overexpressing MDM2 belonged to a group with unfavorable prognostic signs at diagnosis and three of them relapsed or died within 6 months after diagnosis.
TP53基因的突变是多种肿瘤中最常见的分子改变之一，但在儿童和成人血液系统恶性肿瘤中却很少发生。蛋白质积累通常源于导致p53蛋白失活的突变。p53蛋白功能失活的其他原因包括通过MDM2等蛋白质使p53稳定，MDM2是一种能够与p53形成特定复合物的癌蛋白。在本研究中，采用免疫组织化学方法对23例1 - 13岁急性淋巴细胞白血病患者诊断时骨髓样本中的MDM2和p53蛋白表达进行了研究。仅在1例中检测到p53蛋白过表达，而在5例患者的样本中检测到MDM2过表达。所有5例MDM2过表达的患者在诊断时均属于预后不良征象组，其中3例在诊断后6个月内复发或死亡。
