WT-1 is required for early kidney development

  title={WT-1 is required for early kidney development},
  author={Jordan A. Kreidberg and Hannu Sariola and Janet M. Loring and Masahiro Maeda and Jerry Pelletier and David E. Housman and Rudolf Jaenisch},
WT1 and kidney progenitor cells
Recent advances in the understanding of kidney progenitor cells, and the recent identification of WT1 target genes in these cells are discussed.
The Wilms tumor gene, Wt1, is required for Sox9 expression and maintenance of tubular architecture in the developing testis
It is shown that Wt1 is essential for the maintenance of Sertoli cells and seminiferous tubules in the developing testes and that WT1 regulates Sox9, either directly or indirectly, after Sry expression ceases.
The functions of Wt1 in mouse gonad development and somatic cells differentiation
Wt1 is indispensable for somatic cell differentiation and gonad development at different developmental stages, and recent studies find that Wt1 plays important roles in lineage specification and maintenance of gonad somatic cells.
Localization of the Wilms' tumour protein WT1 in avian embryos
It is suggested that WT1 is not required for the epithelial-mesenchymal transition of the coelomic mesothelium, but it might be a marker of the mesothelial-derived cells, where this protein would be acting as a repressor of the differentiation.
Wt1 functions in the development of germ cells in addition to somatic cell lineages of the testis.
Wilms' tumor suppressor gene is involved in the development of disparate kidney forms: Evidence from expression in the Xenopus Pronephros
  • T. Carroll, P. D. Vize
  • Biology
    Developmental dynamics : an official publication of the American Association of Anatomists
  • 1996
The WT1 gene is expressed in a similar temporal and spatial pattern in the vascularized portion of the amphibian pronephroi and in the mammalian metanephroi, arguing that it probably plays a similar crucial role in the morphogenesis of these very different kidney forms.
Gene targeting in kidney development.
Gene targeting of the WT1 gene demonstrated the requirement for this gene product during early urogenital development, and several other genes, including Wnt-4, c-ret, ld and lim 1, have been shown by gene targeting to also be involved in early kidney development.
Pax-2, kidney development, and oncogenesis.
  • G. Dressler
  • Biology, Medicine
    Medical and pediatric oncology
  • 1996
The development of a complex tissue from a few simple precursor cells requires the precise activation and repression of tissue-specific genes that determine cell lineages, tissue patterning, and


Expression of the Wilms' tumor gene WT1 in the murine urogenital system.
Regulation of WT1 expression during development of the gonads is demonstrated, WT1 is a nuclear protein, implicate WT1 in genitourinary development, and a molecular framework toward understanding genitouringinary defects observed among hereditary cases of Wilms' tumor is provided.
WT1 mutations contribute to abnormal genital system development and hereditary Wilms' tumour
Constitutional mutations within the WT1 genes of two individuals with a combination of WT and genital abnormalities are reported as evidence of a role for a recessive oncogene in mammalian development.
Deregulation of Pax-2 expression in transgenic mice generates severe kidney abnormalities
Deregulating the expression of the mouse Pax-2 gene results in histologically abnormal and dysfunctional renal epithelium with properties similar to congenital nephrotic syndrome and repression of Pax-1 is required for normal kidney development and persistent expression of Pax1 may restrict the differentiation potential of renal epithelial cells.
Isolation, characterization, and expression of the murine Wilms' tumor gene (WT1) during kidney development.
The narrow window of time during which WT1 is expressed at high levels in the kidney is consistent with the origin of Wilms' tumor from primitive nephroblasts and the postulated role of this gene as a negative regulator of growth.
Mice deficient for Rb are nonviable and show defects in neurogenesis and haematopoiesis
Transfer of the human retinoblastoma (RB) mini-transgene into the mutant mice corrects the developmental defects and shows that Rb is essential for normal mouse development.
Effects of an Rb mutation in the mouse
A mouse strain has been constructed in which one allele of Rb is disrupted, and heterozygous animals are not predisposed to retinoblastoma, but some display pituitary tumours arising from cells in which the wild-type Rb allele is absent.
Evidence for WT1 as a Wilms tumor (WT) gene: intragenic germinal deletion in bilateral WT.
DNA from both normal and tumor tissue from WT patients is analyzed and a patient with bilateral WT who is heterozygous for a small germinal deletion within this candidate gene is reported, supporting the identification of this locus as an 11p13 WT gene (WT1) and providing direct molecular data supporting the two-hit mutational model for WT.
Role for the Wilms tumor gene in genital development?
The molecular detection of a cytogenetically invisible 1-megabase deletion in an individual with aniridia, cryptorchidism, and hypospadias but no Wilms tumor but no WT implies that the WT gene exerts pleiotropic effect on both kidney and genitourinary development.
A mouse model of the aniridia-Wilms tumor deletion syndrome.
It is suggested that the Small-eye defect is genetically equivalent to human aniridia, but that loss of the murine homolog of the Wilms tumor gene is not sufficient for tumor initiation.