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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
6415
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pubmed:dateCreated |
1993-4-6
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pubmed: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.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Mar
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pubmed:issn |
0028-0836
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
4
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pubmed:volume |
362
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pubmed:geneSymbol |
Pax-2
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
65-7
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pubmed:dateRevised |
2008-11-21
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pubmed:meshHeading |
pubmed-meshheading:8383297-Animals,
pubmed-meshheading:8383297-Blood Urea Nitrogen,
pubmed-meshheading:8383297-Cadherins,
pubmed-meshheading:8383297-Cytomegalovirus,
pubmed-meshheading:8383297-DNA-Binding Proteins,
pubmed-meshheading:8383297-Female,
pubmed-meshheading:8383297-Gene Expression Regulation,
pubmed-meshheading:8383297-Genes, Viral,
pubmed-meshheading:8383297-Humans,
pubmed-meshheading:8383297-Immunohistochemistry,
pubmed-meshheading:8383297-Kidney,
pubmed-meshheading:8383297-Male,
pubmed-meshheading:8383297-Mice,
pubmed-meshheading:8383297-Mice, Transgenic,
pubmed-meshheading:8383297-PAX2 Transcription Factor,
pubmed-meshheading:8383297-Potassium,
pubmed-meshheading:8383297-Promoter Regions, Genetic,
pubmed-meshheading:8383297-Reference Values,
pubmed-meshheading:8383297-Transcription Factors
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pubmed:year |
1993
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pubmed:articleTitle |
Deregulation of Pax-2 expression in transgenic mice generates severe kidney abnormalities.
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pubmed:affiliation |
Laboratory of Mammalian Genes and Development, National Institute of Child Health and Human Development, Bethesda, Maryland 20892.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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