Deregulation of Pax-2 expression in transgenic mice generates severe kidney abnormalities (original) (raw)

Nature volume 362, pages 65–67 (1993)Cite this article

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.

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Author notes

  1. J. Erby Wilkinson: Department of Pathobiology, College of Veterinary Medicine, University of Tennessee, Knoxville, Tennessee 37901, USA

Authors and Affiliations

  1. Laboratory of Mammalian Genes and Development, National Institute of Child Health and Human Development, Bethesda, Maryland, 20892, USA
    Gregory R. Dressler, J. Erby Wilkinson, Uwe W. Rothenpieler, Larry T. Patterson, Lisa Williams-Simons & Heiner Westphal

Authors

  1. Gregory R. Dressler
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  2. J. Erby Wilkinson
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  3. Uwe W. Rothenpieler
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  4. Larry T. Patterson
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  5. Lisa Williams-Simons
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  6. Heiner Westphal
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Dressler, G., Wilkinson, J., Rothenpieler, U. et al. Deregulation of Pax-2 expression in transgenic mice generates severe kidney abnormalities.Nature 362, 65–67 (1993). https://doi.org/10.1038/362065a0

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