Wilms' tumour is a paediatric kidney malignancy that arises through ab
errant differentiation of nephric stem cells. We are studying the role
of one Wilms' tumour predisposition gene, WT1. This is a tumour suppr
essor gene whose function is required for normal development of the ge
nitourinary system. WT1 encodes a putative transcriptional repressor o
f the zinc finger family. Here we discuss how one of the normal functi
ons of WT1 may be to suppress myogenesis during kidney development. Fu
rthermore, we describe how we are proposing to use YAC (yeast artifici
al 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 genitourinar
y evolution.