Document Detail


WT1 is a modifier of the Pax2 mutant phenotype: cooperation and interaction between WT1 and Pax2.
MedLine Citation:
PMID:  14603255     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Metanephric kidney development requires an inductive interaction between the ureteric bud and progenitor mesenchyme, where the early expression of two genes, Wilms' tumour 1 (WT1) and paired box 2 (Pax2), establishes critical but unknown developmental pathways. Indeed, transgenic mice with deregulated overexpression of Pax2 exhibit structural kidney defects and impaired renal function, as do mice harboring targeted disruptions and/or spontaneous mutations of either the Pax2 or WT1 genes. WT1 and Pax2 are thought to regulate each other's expression during renal development. To better define the relationship between WT1 and Pax2, we generated mouse embryos containing heterozygous mutations in both genes. WT1(+/-)/Pax2(1Neu/+) kidneys were 50% smaller than wild-type kidneys. They were characterized by severe attenuation of the renal medulla, and reduced development of calyces and the renal pelvis. Renal cortex development in compound heterozygotes culminated in fewer nephrons than in WT1(+/-), Pax2(1Neu/+) or wild-type mice. Only minor variations in the mesenchymal expression pattern of Pax2 protein, and the mRNA expression levels of Pax2 and WT1, were noted in mutant kidneys. We show that WT1 and Pax2 proteins interact in vitro and in vivo, demonstrating that WT1 and Pax2 can form a molecular complex. Our data suggest that WT1 is a modifier of the Pax2 mutant phenotype.
Authors:
Maria Teresa Discenza; Shujie He; Tae Ho Lee; Lee Lee Chu; Brad Bolon; Paul Goodyer; Michael Eccles; Jerry Pelletier
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Oncogene     Volume:  22     ISSN:  0950-9232     ISO Abbreviation:  Oncogene     Publication Date:  2003 Nov 
Date Detail:
Created Date:  2003-11-06     Completed Date:  2003-12-11     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8711562     Medline TA:  Oncogene     Country:  England    
Other Details:
Languages:  eng     Pagination:  8145-55     Citation Subset:  IM    
Affiliation:
Department of Biochemistry, McGill University, Montreal, Quebec, Canada.
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MeSH Terms
Descriptor/Qualifier:
Animals
DNA-Binding Proteins / genetics*,  metabolism
Kidney / embryology
Mice
Mice, Inbred C57BL
Mutation*
PAX2 Transcription Factor
Transcription Factors / genetics*,  metabolism
WT1 Proteins / metabolism*
Chemical
Reg. No./Substance:
0/DNA-Binding Proteins; 0/PAX2 Transcription Factor; 0/Pax2 protein, mouse; 0/Transcription Factors; 0/WT1 Proteins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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