THE WILMS-TUMOR SUPPRESSOR WT1 - APPROACHES TO GENE-FUNCTION

Occurring with a frequency of 1 in 10,000 live births, Wilms' tumor is one of the most common solid tumors of children. The genetic basis of this tumor is highly complex and several loci have been shown to be a ssociated with tumor formation. Thus far, however, WT1 is the only gen e that has been isolated and proven to carry mutations within Wilms' t umors. During the last few years, a wealth of experiments has been car ried out to address the function of WT1 as a tumor suppressor and deve lopmental regulator. This review focuses on studies addressing WT1 fun ction; new approaches to understand WT1 function in vivo and present t ransgenic data in which WT1 was driven ectopically using a CMV promote r are discussed. Our results suggest that ubiquitous expression of WT1 is not compatible with embryonic development.