The development of a complex tissue from a few simple precursor cells
requires the precise activation and repression of tissue-specific gene
s that determine cell lineages, tissue patterning, and cellular prolif
eration. In the kidney, a number of recently identified genes are crit
ical for normal development. Among these, the Pax-2 gene encodes a tra
nscription factor that is expressed in the ureter bud, in the induced
kidney mesenchyme, and in the progenitor cells of the glomerular and t
ubular epithelium. Although the differentiation of the renal epitheliu
m requires Pax-2 function, failure to suppress the gene in mature epit
helium is detrimental to normal renal function. Recent, data suggest t
hat the Wilms' tumor-suppressor gene WT1 can down-regulate Pax-2 expre
ssion, consistent with high levels of Pax-2 in Wilms' tumors. Addition
al studies suggest that reactivation of this developmental regulator c
an contribute to a variety of other renal diseases. (C) 1996 Wiley-Lis
s, Inc.