The candidate Wilms' tumour gene is involved in genitourinary development
- PMID:2164159
- DOI: 10.1038/346194a0
The candidate Wilms' tumour gene is involved in genitourinary development
Abstract
Wilms' tumour is an embryonic kidney tumour thought to arise through aberrant mesenchymal stem cell differentiation and to result from loss of function of a 'tumour suppressor' gene(s). Both sporadic and syndrome-associated Wilms' tumours are accompanied by an increased frequency of abnormalities of the urinary tract and genitalia. Deletional analysis of individuals with the WAGR syndrome (for, Wilms' tumour, aniridia, genitourinary abnormalities and mental retardation) showed that a Wilms' tumour gene lies at chromosomal position 11p13. This led to the isolation of a candidate Wilms' tumour gene, encoding a zinc-finger protein which is likely to be a transcription factor. To gain insight into the role of this candidate gene in normal development and tumorigenesis, we have now performed in situ messenger RNA hybridization on sections of human embryos and Wilms' tumours. The candidate Wilms' tumour gene is expressed specifically in the condensed mesenchyme, renal vesicle and glomerular epithelium of the developing kidney, in the related mesonephric glomeruli and in cells approximating these structures in tumours. The other main sites of expression are the genital ridge, fetal gonad and mesothelium. These data suggest that (1) this candidate is indeed a Wilms' tumour gene, (2) the associated genital abnormalities are pleiotropic effects of mutation in the Wilms' tumour gene itself, in support of recent genetic analysis, and (3) this gene has a specific role in kidney development and a wider role in mesenchymal-epithelial transitions.
Similar articles
- Cell types expressing the Wilms' tumour gene (WT1) in Wilms' tumours: implications for tumour histogenesis.Pritchard-Jones K, Fleming S.Pritchard-Jones K, et al.Oncogene. 1991 Dec;6(12):2211-20.Oncogene. 1991.PMID:1722569
- Wilms' tumour as a paradigm for the relationship of cancer to development.Pritchard-Jones K, Hastie ND.Pritchard-Jones K, et al.Cancer Surv. 1990;9(3):555-78.Cancer Surv. 1990.PMID:1966278Review.
- Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping.Gessler M, Poustka A, Cavenee W, Neve RL, Orkin SH, Bruns GA.Gessler M, et al.Nature. 1990 Feb 22;343(6260):774-8. doi: 10.1038/343774a0.Nature. 1990.PMID:2154702
- Inactivation of the remaining allele of the WT1 gene in a Wilms' tumour from a WAGR patient.Brown KW, Watson JE, Poirier V, Mott MG, Berry PJ, Maitland NJ.Brown KW, et al.Oncogene. 1992 Apr;7(4):763-8.Oncogene. 1992.PMID:1314370
- Wilms tumour: a developmental anomaly.Hastie ND, Bickmore W, Pritchard-Jones K, Porteous DJ, van Heyningen V.Hastie ND, et al.Princess Takamatsu Symp. 1989;20:145-50.Princess Takamatsu Symp. 1989.PMID:2562179Review.
Cited by
- Denys-Drash syndrome associated WT1 glutamine 369 mutants have altered sequence-preferences and altered responses to epigenetic modifications.Hashimoto H, Zhang X, Zheng Y, Wilson GG, Cheng X.Hashimoto H, et al.Nucleic Acids Res. 2016 Dec 1;44(21):10165-10176. doi: 10.1093/nar/gkw766. Epub 2016 Sep 4.Nucleic Acids Res. 2016.PMID:27596598Free PMC article.
- Genome-wide association studies for pelvic organ prolapse in the Japanese population.Matsunami M, Imamura M, Ashikari A, Liu X, Tomizuka K, Hikino K, Miwa K, Kadekawa K, Suda T; Biobank Japan project; Matsuda K, Miyazato M, Terao C, Maeda S.Matsunami M, et al.Commun Biol. 2024 Sep 30;7(1):1188. doi: 10.1038/s42003-024-06875-2.Commun Biol. 2024.PMID:39349682Free PMC article.
- Isolation, characterization, and expression of the murine Wilms' tumor gene (WT1) during kidney development.Buckler AJ, Pelletier J, Haber DA, Glaser T, Housman DE.Buckler AJ, et al.Mol Cell Biol. 1991 Mar;11(3):1707-12. doi: 10.1128/mcb.11.3.1707-1712.1991.Mol Cell Biol. 1991.PMID:1671709Free PMC article.
- Pax-2 is a DNA-binding protein expressed in embryonic kidney and Wilms tumor.Dressler GR, Douglass EC.Dressler GR, et al.Proc Natl Acad Sci U S A. 1992 Feb 15;89(4):1179-83. doi: 10.1073/pnas.89.4.1179.Proc Natl Acad Sci U S A. 1992.PMID:1311084Free PMC article.
- Frasier and Denys-Drash syndromes: different disorders or part of a spectrum?Koziell A, Grundy R.Koziell A, et al.Arch Dis Child. 1999 Oct;81(4):365-9. doi: 10.1136/adc.81.4.365.Arch Dis Child. 1999.PMID:10490448Free PMC article.Review.No abstract available.
MeSH terms
Substances
Related information
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical