Pearson P L, Van der Luijt R B
Department of Human Genetics, Utrecht University, The Netherlands.
J Intern Med. 1998 Jun;243(6):413-7. doi: 10.1046/j.1365-2796.1998.00343.x.
During the past two decades an overwhelming amount of knowledge has been acquired on the molecular genetics of human cancer. It is now evident that cancer is essentially a genetic disease, arising from inherited and/or somatically acquired mutations at different genetic loci, and that tumourigenesis is a multistep process. Gene mapping studies of inherited cancer syndromes have resulted in the identification of many genes implicated in the initiation of tumours. Importantly, alterations of the same genes were also found to play a role in the development of common, non-familial tumours. The genes involved belong to distinct functional classes, and include proto-oncogenes and tumour suppressor genes, which are regulators of cellular growth and proliferation, cell adhesion and programmed cell death. Another class of cancer susceptibility genes consists of DNA repair genes, which are involved in maintaining genomic stability. In unravelling the genetic basis of cancer, the localization and identification of genes involved in tumourigenesis can be considered as the 'easy' part; determination of the normal physiological function of these genes and their precise role in tumourigenesis has proved to be much more difficult. In this review, we highlight some of the major breakthroughs in the field of cancer genetics, and discuss recent insights in the putative role of proto-oncogenes, tumour suppressor genes and DNA repair genes in the initiation and progression of cancer. Also, we point to some of the challenges to be faced in the coming years.
在过去二十年中,我们在人类癌症分子遗传学方面积累了大量知识。现在很明显,癌症本质上是一种基因疾病,由不同基因位点的遗传和/或体细胞获得性突变引起,并且肿瘤发生是一个多步骤过程。对遗传性癌症综合征的基因定位研究已经确定了许多与肿瘤起始相关的基因。重要的是,同样这些基因的改变也被发现参与了常见的非家族性肿瘤的发展。所涉及的基因属于不同的功能类别,包括原癌基因和肿瘤抑制基因,它们是细胞生长和增殖、细胞黏附以及程序性细胞死亡的调节因子。另一类癌症易感基因由DNA修复基因组成,它们参与维持基因组稳定性。在揭示癌症的遗传基础时,肿瘤发生相关基因的定位和鉴定可以被认为是“容易”的部分;而确定这些基因的正常生理功能及其在肿瘤发生中的精确作用则要困难得多。在这篇综述中,我们重点介绍癌症遗传学领域的一些重大突破,并讨论原癌基因、肿瘤抑制基因和DNA修复基因在癌症起始和进展中假定作用的最新见解。此外,我们还指出了未来几年将要面临的一些挑战。