WT1 is a modifier of the Pax2 mutant phenotype: cooperation and interaction between WT1 and Pax2.

作者: Maria Teresa Discenza , Shujie He , Tae Ho Lee , Lee Lee Chu , Brad Bolon

DOI: 10.1038/SJ.ONC.1206997

关键词:

摘要: Metanephric kidney development requires an inductive interaction between the ureteric bud and progenitor mesenchyme, where early expression of two genes, Wilms’ tumour 1 (WT1) paired box 2 (Pax2), establishes critical but unknown developmental pathways. Indeed, transgenic mice with deregulated overexpression Pax2 exhibit structural defects impaired renal function, as do harboring targeted disruptions and/or spontaneous mutations either or WT1 genes. are thought to regulate each other's during development. To better define relationship Pax2, we generated mouse embryos containing heterozygous in both WT1+/−/Pax21Neu/+ kidneys were 50% smaller than wild-type kidneys. They characterized by severe attenuation medulla, reduced calyces pelvis. Renal cortex compound heterozygotes culminated fewer nephrons WT1+/−, Pax21Neu/+ mice. Only minor variations mesenchymal pattern protein, mRNA levels WT1, noted mutant We show that proteins interact vitro vivo, demonstrating can form a molecular complex. Our data suggest is modifier phenotype.

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