Wilms' tumor suppressor gene WT1: From structure to renal pathophysiologic features

Journal of the American Society of Nephrology

Volumn 11, Issue SUPPL. 16, 2000, Pages

  Mrowka, C ^a   Schedl, A ^a  

^a MAX DELBRÜCK CENTER FOR MOLECULAR MEDICINE   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

CANCER SURVIVAL; DENYS DRASH SYNDROME; GENE STRUCTURE; HUMAN; NEPHROBLASTOMA; PATHOPHYSIOLOGY; PRIORITY JOURNAL; REVIEW; TUMOR SUPPRESSOR GENE;

EID: 0033764489     PISSN: 10466673     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Review

References (69)

1. Organogenesis of the kidney
2. Molecular development of the kidney: A review of the results of gene disruption studies
3. Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus
4. Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping
5. A non-AUG translational initiation event generates novel WT1 isoforms
6. Internal translation initiation generates novel WT1 protein isoforms with distinct biological properties
7. Alternative splicing and genomic structure of the Wilms tumor gene WT
8. RNA editing in the Wilms' tumor susceptibility gene, WT1
9. The Wilms' tumor suppressor WT1: Approaches to gene function
10. WT1 suppresses synthesis of the epidermal growth factor receptor and induces apoptosis
11. Pax2, a new murine paired-box-containing gene and its expression in the developing excretory system
12. Repression of the insulin-like growth factor II gene by the Wilms tumor suppressor WT1
13. The Wilms tumor suppressor WT1 encodes a transcriptional activator of amphiregulin
14. The evolution of WT1 sequence and expression pattern in the vertebrates
15. Complete sequencing of the Fugu WAGR region from WT1 to PAX6: Dramatic compaction and conservation of synteny with human chromosome 11p13
16. Differential function of Wilms' tumor gene WT1 splice isoforms in transcriptional regulation
17. RNA binding by the Wilms tumor suppressor zinc finger proteins
18. An RNA recognition motif in Wilms' tumour protein (WT1) revealed by structural modelling
19. Subnuclear localization of WT1 in splicing or transcription factor domains is regulated by alternative splicing
20. WT1 interacts with the splicing factor U2AF65 in an isoform-dependent manner and can be incorporated into spliceosomes
21. + ribonucleoprotein
22. Association d'un syndrome anatomo-pathologique de pseudo-hermaphrodisme masculin, d'une tumeur de Wilms, d'une nephropathie parenchymateuse et d'un mosaicisme XX/XY
23. A syndrome of pseudohermaphroditism, Wilms' tumor, hypertension, and degenerative renal disease
24. Identification of constitutional WT1 mutations, in patients with isolated diffuse mesangial sclerosis, and analysis of genotype/phenotype correlations by use of a computerized mutation database
25. WT1 and PAX-2 podocyte expression in Denys-Drash syndrome and isolated diffuse mesangial sclerosis
26. Unexpected Wilm's tumor in a pediatric renal transplant recipient: Suspected Denys-Drash syndrome
27. Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome
28. WT1: Renal development and glomerulopathies
29. Antagonism of WT1 activity by protein self-association
30. A zinc finger truncation of murine WT1 results in the characteristic urogenital abnormalities of Denys-Drash syndrome
31. Gonadoblastoma associated with pure gonadal dysgenesis in monozygotic twins
32. Donor splice-site mutations in WT1 are responsible for Frasier syndrome
33. Exon 9 mutations in the WT1 gene, without influencing KTS splice isoforms, are also responsible for Frasier syndrome
34. The same mutation affecting the splicing of WT1 gene is present in Frasier syndrome patients with or without Wilms' tumor
35. Diffuse mesangial sclerosis: A congenital glomerulopathy with nephrotic syndrome
36. Spectrum of early onset nephrotic syndrome associated with WT1 missense mutations
37. Frasier syndrome: A cause of segmental glomerulosclerosis in a 46,XX female
38. Frasier syndrome is caused by defective alternative splicing of WT1 leading to an altered ratio of WT1+/-KTS splice isoforms
39. Inactivation of WT1 in nephrogenic rests, genetic precursors to Wilms' tumour
40. Chromosomal imbalance in the aniridia-Wilms' tumor association: 11p interstitial deletion
41. Complete physical map of the WAGR region of 11p13 localizes a candidate Wilms' tumor gene
42. Positional cloning and characterisation of a paired box- and homeobox-containing gene from the aniridia region
43. The reticulocalbindin gene maps to the WAGR region in human and to the small eye Harwell deletion in mouse
44. An internal deletion within an 11p13 zinc finger gene contributes to the development of Wilms' tumor
45. Evidence for WT1 as a Wilms tumor (WT) gene: Intragenic germinal deletion in bilateral WT
46. The candidate Wilms' tumour gene is involved in genitourinary development
47. Isolation, characterization, and expression of the murine Wilms' tumor gene (WT1) during kidney development
48. Initial differentiation of the metanephric mesenchyme is independent of WT1 and the ureteric bud
49. The expression of the Wilms' tumour gene, WT1, in the developing mammalian embryo
50. Factors controlling growth, motility, and morphogenesis of normal and malignant epithelial cells
51. Expression of WT1 protein in fetal kidneys and Wilms tumors
52. The WT1 Wilms tumor gene product: A developmentally regulated transcription factor in the kidney that functions as a tumor suppressor
53. The Wilms tumor suppressor gene WT1 is required for development of the spleen
54. Wilms' tumor 1 and Dax-1 modulate the orphan nuclear receptor SF-1 in sex-specific gene expression
55. WT-1 is required for early kidney development
56. YAC complementation shows a requirement for Wt1 in the development of epicardium, adrenal gland and throughout nephrogenesis
57. Pax in development
58. Deregulation of Pax-2 expression in transgenic mice generates severe kidney abnormalities
59. Pax-2 controls multiple steps of urogenital development
60. The mouse Pax2 (1Neu) mutation is identical to a human PAX2 mutation in a family with renal-coloboma syndrome and results in developmental defects of the brain, ear, eye, and kidney
61. Repression of Pax-2 by WT1 during normal kidney development
62. Differential regulation of the human Wilms tumour suppressor gene (WT1) promoter by two isoforms of PAX2
63. The paired-box transcription factor, PAX2, positively modulates expression of the Wilms' tumor suppressor gene (WT1)
64. Recent advances in mammalian RNA editing
65. Analysis of possible WT1 RNA processing in primary Wilms tumors
66. Modulation of DNA binding specificity by alternative splicing of the Wilms tumor wt1 gene transcript
67. WT1, the Wilms' tumor suppressor gene product, represses transcription through an interactive nuclear protein
68. A structure-function analysis of transcriptional repression mediated by the WT1, Wilms' tumor suppressor protein
69. WT1-mediated transcriptional activation is inhibited by dominant negative mutant proteins