DEREGULATION OF PAX-2 EXPRESSION IN TRANSGENIC MICE GENERATES SEVERE KIDNEY ABNORMALITIES
DEREGULATION OF PAX-2 EXPRESSION IN TRANSGENIC MICE GENERATES SEVERE KIDNEY ABNORMALITIES
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DOI：
10.1038/362065a0
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发表时间：
1993-03-04
期刊：
影响因子：
64.8
通讯作者：
WESTPHAL, H
中科院分区：
文献类型：
--
作者：
DRESSLER, GR;WILKINSON, JE;WESTPHAL, H
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.
