| First Author | Dressler GR | Year | 1993 |
| Journal | Nature | Volume | 362 |
| Issue | 6415 | Pages | 65-7 |
| PubMed ID | 8383297 | Mgi Jnum | J:4199 |
| Mgi Id | MGI:52697 | Doi | 10.1038/362065a0 |
| Citation | Dressler GR, et al. (1993) Deregulation of Pax-2 expression in transgenic mice generates severe kidney abnormalities. Nature 362(6415):65-7 |
| abstractText | The Pax genes comprise a family of transcription factors active in specific tissues during embryonic development and are associated with at least three developmental mutations in mouse and man. In the developing kidney, Pax-2 is expressed in the induced mesenchyme, in the ureter epithelium, and in early epithelial structures derived from the mesenchyme. Pax-2 expression is repressed upon terminal differentiation of the renal tubule epithelium, but persists in the undifferentiated epithelium of human Wilms' tumours. We have produced a dominant gain-of-function mutation in transgenic mice by deregulating the expression of the mouse Pax-2 gene. The data obtained with four independently derived transgenic embryos and with one transgenic line demonstrate that deregulated Pax-2 expression results in histologically abnormal and dysfunctional renal epithelium with properties similar to congenital nephrotic syndrome. Thus, repression of Pax-2 is required for normal kidney development and persistent expression of Pax-2 may restrict the differentiation potential of renal epithelial cells. |
