| | |  | | | | Record from Web of Science® | | | | | | |
| DEREGULATION OF PAX-2 EXPRESSION IN TRANSGENIC MICE GENERATES SEVERE KIDNEY ABNORMALITIES |
|
|
| Author(s): DRESSLER GR, WILKINSON JE, ROTHENPIELER UW, PATTERSON LT, WILLIAMSSIMONS L, WESTPHAL H |
| Source: NATURE Volume: 362 Issue: 6415 Pages: 65-67 Published: MAR 4 1993 |
| Times Cited: 202 References: 16 |
| Abstract: 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 man1,2. In the developing kidney, Pax-2 is expressed in the induced mesenchyme, in the ureter epithelium, and in early epithelial structures derived from the mesenchyme3. Pax-2 expression is repressed upon terminal differentiation of the renal tubule epithelium, but persists in the undifferentiated epithelium of human Wilms' tumours4,5. 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. |
| Document Type: Article |
| Language: English |
| Reprint Address: DRESSLER, GR (reprint author), NICHHD, MAMMALIAN GENES & DEV LAB, BETHESDA, MD 20892 USA |
Addresses:
1. UNIV TENNESSEE, COLL VET MED, DEPT PATHOBIOL, KNOXVILLE, TN 37901 USA |
| Publisher: MACMILLAN MAGAZINES LTD, PORTERS SOUTH, 4 CRINAN ST, LONDON, ENGLAND N1 9XW |
| Subject Category: Multidisciplinary Sciences |
| IDS Number: KP976 |
| ISSN: 0028-0836 |
|
| | | | | | | | | Record from Web of Science® | | | | | | | | | |