Miyagawa K, Kent J, Schedl A, van Heyningen V, Hastie N D
MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK.
J Cell Sci Suppl. 1994;18:1-5. doi: 10.1242/jcs.1994.supplement_18.1.
Wilms' tumour is a paediatric kidney malignancy that arises through aberrant differentiation of nephric stem cells. We are studying the role of one Wilms' tumour predisposition gene, WT1. This is a tumour suppressor gene whose function is required for normal development of the genitourinary system. WT1 encodes a putative transcriptional repressor of the zinc finger family. Here we discuss how one of the normal functions of WT1 may be to suppress myogenesis during kidney development. Furthermore, we describe how we are proposing to use YAC (yeast artificial chromosome) transgenesis to analyse WT1 regulation and function in mice. We also discuss the evolution of the WT1 gene amongst different vertebrate classes and how this may provide insights into genitourinary evolution.
肾母细胞瘤是一种起源于肾干细胞异常分化的小儿肾脏恶性肿瘤。我们正在研究一种肾母细胞瘤易感基因WT1的作用。这是一种肿瘤抑制基因,其功能对于泌尿生殖系统的正常发育是必需的。WT1编码一种锌指家族的假定转录抑制因子。在此我们讨论WT1的正常功能之一可能是在肾脏发育过程中抑制肌发生。此外,我们描述了我们计划如何利用酵母人工染色体转基因技术来分析WT1在小鼠中的调控和功能。我们还讨论了WT1基因在不同脊椎动物类群中的进化情况以及这如何为泌尿生殖系统的进化提供见解。