AC T00899
XX
ID T00899
XX
DT 10.11.1992 (created); ewi.
DT 25.11.2013 (updated); vad.
CO Copyright (C), QIAGEN.
XX
FA WT1
XX
SY Swiss-Prot isoform-1; Wilm's tumor protein; Wilms tumor zinc finger protein; WT-ZFP; WT1 +KTS; WT1(+/+); WT33.
XX
OS human, Homo sapiens
OC eukaryota; animalia; metazoa; chordata; vertebrata; tetrapoda; mammalia; eutheria; primates
XX
GE G004120 WT1; HGNC: WT1.
XX
CL C0001; CH; 2.3.3.0.21.1.
XX
SZ 449 AA; 49.2 kDa (cDNA) (calc.).
XX
SQ MGSDVRDLNALLPAVPSLGGGGGCALPVSGAAQWAPVLDFAPPGASAYGSLGGPAPPPAP
SQ PPPPPPPPHSFIKQEPSWGGAEPHEEQCLSAFTVHFSGQFTGTAGACRYGPFGPPPPSQA
SQ SSGQARMFPNAPYLPSCLESQPAIRNQGYSTVTFDGTPSYGHTPSHHAAQFPNHSFKHED
SQ PMGQQGSLGEQQYSVPPPVYGCHTPTDSCTGSQALLLRTPYSSDNLYQMTSQLECMTWNQ
SQ MNLGATLKGVAAGSSSSVKWTEGQSNHSTGYESDNHTTPILCGAQYRIHTHGVFRGIQDV
SQ RRVPGVAPTLVRSASETSEKRPFMCAYPGCNKRYFKLSHLQMHSRKHTGEKPYQCDFKDC
SQ ERRFSRSDQLKRHQRRHTGVKPFQCKTCQRKFSRSDHLKTHTRTHTGKTSEKPFSCRWPS
SQ CQKKFARSDELVRHHNMHQRNMTKLQLAL
XX
SC Swiss-Prot#P19544-1
XX
FT 1 321 
PF02165; Wilm's tumour protein.
FT 250 266 
missing in splice variant I, seems to support repressor and cell cycle blocking activity [9].
FT 323 347 
PF00096; zf-C2H2.
FT 323 347 
SM00355; c2h2final6.
FT 323 352 
PS50157; ZINC_FINGER_C2H2_2.
FT 353 377 
PF00096; zf-C2H2.
FT 353 377 
SM00355; c2h2final6.
FT 353 382 
PS50157; ZINC_FINGER_C2H2_2.
FT 383 405 
PF00096; zf-C2H2.
FT 383 405 
SM00355; c2h2final6.
FT 383 410 PS50157; ZINC_FINGER_C2H2_2.
FT 408 410 missing in splice variant -KTS, may influence the type of nuclear distribution [10].
FT 414 438 PF00096; zf-C2H2.
FT 414 438 SM00355; c2h2final6.
FT 414 443 PS50157; ZINC_FINGER_C2H2_2.
XX
SF 4 zinc finger motifs;
SF full-length protein with several splice variants;
SF physical association with p53 [13];
SF but: no interaction with p53 in yeast two-hybrid assay [16];
XX
CP expressed in cells that undergo a mesenchymal to epithelial switch and belong to tissues of mesodermal origin [10]; fetal kidney, testis and ovary [9] [9] [10].
XX
FF repressor of EGR-1 sites through proline-rich N-terminus [6];
FF mapped to chromosome region 11p13 [2];
FF mutations may cause Wilms tumor (nephroblastoma);
FF somatic mutations of the zinc finger motifs were found in cases of sporadic Wilms tumor [15];
FF necessary for normal early urogenital development [9];
FF fused to EWS (Ewing sarcoma) in t(11;
FF 22)(p13;
FF q12), a translocation associated with desmoplastic small round cell tumor [12];
FF WT1 function and the regulation of cell cycle-dependent kinases (CDK2, CDK4) may be linked [9];
FF associates with specific components of the splicing machinery like isoform T01840 [10];
FF antiproliferative effect may be mediated by repression of insulin-like growth factor I receptor (IGF-I-R) gene receptor [11];
FF does not repress IGF-I-R in Saos-2 or U2OS cells [17];
FF transcriptional repressor of EGF receptor promoter [17];
FF binding to RNA via zinc finger [10];
FF expression stabilizes and partially inactivate p53 [16];
XX
IN T00671 p53; human, Homo sapiens.
IN T04997 p53; rat, Rattus norvegicus.
IN T06006 p73alpha; monkey, Cercopithecus aethiops.
XX
MX M07436 V$WT1_Q4.
MX M01118 V$WT1_Q6.
MX M02036 V$WT1_Q6_01.
MX M03893 V$WT1_Q6_02.
XX
BS R04849.
BS R58960.
BS R33938.
BS R33946.
BS R33947.
BS R05048.
BS R02313.
BS R02314.
BS R02315.
BS R04700.
BS R04733.
BS R01615.
BS R31231.
BS R31233.
BS R31234.
BS R04858.
BS R04859.
BS R04861.
BS R02262.
XX
DR TRANSPATH: MO000015012.
DR PATHODB: MT010362.
DR PATHODB: MT010363.
DR PATHODB: MT010364.
DR PATHODB: MT010365.
DR PATHODB: MT010366.
DR PATHODB: MT010367.
DR PATHODB: MT010368.
DR PATHODB: MT010369.
DR PATHODB: MT010370.
DR PATHODB: MT010371.
DR PATHODB: MT010372.
DR PATHODB: MT010373.
DR PATHODB: MT010374.
DR PATHODB: MT010375.
DR PATHODB: MT010376.
DR PATHODB: MT010377.
DR PATHODB: MT010378.
DR PATHODB: MT010379.
DR PATHODB: MT010380.
DR PATHODB: MT010381.
DR PATHODB: MT010382.
DR PATHODB: MT010383.
DR PATHODB: MT010384.
DR PATHODB: MT010385.
DR PATHODB: MT010386.
DR PATHODB: MT010387.
DR PATHODB: MT010388.
DR PATHODB: MT010389.
DR PATHODB: MT010390.
DR PATHODB: MT010391.
DR PATHODB: MT010392.
DR PATHODB: MT010393.
DR PATHODB: MT010394.
DR PATHODB: MT010395.
DR PATHODB: MT010396.
DR PATHODB: MT010397.
DR PATHODB: MT010398.
DR EMBL: M30393;
DR EMBL: M80217;
DR EMBL: M80218;
DR EMBL: M80219;
DR EMBL: M80220;
DR EMBL: M80221;
DR EMBL: M80228;
DR EMBL: M80229;
DR EMBL: M80231;
DR EMBL: M80232;
DR EMBL: X51630;
DR UniProtKB: P19544-1;
XX
RN [1]; RE0000185.
RX PUBMED: 2154335.
RA Call K. W., Glaser T., Ito C. Y., Buckler A. J., Pelletier J., Haber D. A., Rose E. A., Kral A., Yeger H., Housman D. E.
RT Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus
RL Cell 60:509-520 (1990).
RN [2]; RE0001934.
RX PUBMED: 1533441.
RA Jain J., McCaffrey P. G., Valge-Archer V. E., Rao A.
RT Nuclear factor of activated T cells contain Fos and Jun
RL Nature 356:801-804 (1992).
RN [3]; RE0002243.
RX PUBMED: 1662794.
RA Morris J. F., Madden St. L., Tournay O. E., Cook D. M., Sukhatme V. P., Rauscher III F. J.
RT Characterization of the zinc finger protein encoded by the Wilms' tumor locus
RL Oncogene 6:2339-2348 (1991).
RN [4]; RE0002457.
RX PUBMED: 1317572.
RA Little M. H., Prosser J. E., Condie A., Smith P. J., van Heyningen V., Hastie N. D.
RT Zinc finger point mutations within the WT1 gene in Wilms tumor patients
RL Proc. Natl. Acad. Sci. USA 89:4791-4795 (1992).
RN [5]; RE0002657.
RX PUBMED: 2244209.
RA Rauscher F. J., Morris F. J., Tournay O. E., Cook D. M., Curran T.
RT Binding of the Wilms' tumor locus zinc finger protein to the EGR-1 consensus sequence
RL Science 250:1259-1262 (1990).
RN [6]; RE0002658.
RX PUBMED: 1654597.
RA Madden S. L., Cook D. M., Morris J. F., Gashler A., Sukhatme V. P., Rauscher F. J.
RT Transcriptional repression mediated by the WT1 Wilms tumor gene product
RL Science 253:1550-1553 (1991).
RN [7]; RE0002675.
RX PUBMED: 1321494.
RA Bickmore W. A., Oghene K., Little M. H., Seawright A., van Heyningen V., Hastie N. D.
RT Modulation of DNA binding specificity by alternative splicing of the Wilms tumor wt1 gene transcript
RL Science 257:235-237 (1992).
RN [8]; RE0002786.
RX PUBMED: 2163761.
RA Haber D. A., Buckler A. J., Glaser T., Call K. M., Pelletier J., Sohn R. L., Douglass E. C., Housman D. E.
RT Am internal deletion within an 11p13 zinc finger gene contributes to the development of Wilms' tumor
RL Cell 61:1257-1269 (1990).
RN [9]; RE0006497.
RX PUBMED: 7753836.
RA Kudoh T., Ishidate T., Moriyama M., Toyoshima K., Akiyama T.
RT G1 phase arrest induced by Wilms tumor protein WT1 is abrogated by cyclin/CDK complexes
RL Proc. Natl. Acad. Sci. USA 92:4517-4521 (1995).
RN [10]; RE0006498.
RX PUBMED: 7736591.
RA Larsson S. H., Charlieu J.-P., Miyagawa K., Engelkamp D., Rassoulzadegan M., Ross A., Cuzin F., van Heyningen V., Hastie N. D.
RT Subnuclear localization of WT1 in splicing or transcription factor domains is regulated by alternative splicing
RL Cell 81:391-401 (1995).
RN [11]; RE0006499.
RX PUBMED: 7791758.
RA Werner H., Shen-Orr Z., Rauscher III F. J., Morris J. F., Roberts jr C. T., LeRoith D.
RT Inhibition of cellular proliferation by Wilms' tumor suppressor WT1 is associated with suppression of insulin-like growth factor I receptor gene expression
RL Mol. Cell. Biol. 15:3516-3522 (1995).
RN [12]; RE0006506.
RX PUBMED: 7862627.
RA Gerald W. L., Rosai J., Ladanyi M.
RT Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor
RL Proc. Natl. Acad. Sci. USA 92:1028-1032 (1995).
RN [13]; RE0006640.
RX PUBMED: 8393820.
RA Rauscher III F. J.
RT The WT1 Wilms tumor gene product: A developmentally regulated transcription factor in the kidney that functions as a tumor suppressor
RL FASEB J. 7:896-903 (1993).
RN [14]; RE0006643.
RX PUBMED: 1671709.
RA Buckler A. J., Pelletier J., Haber D. A., Glaser T., Housman D.
RT Isolation, characterization, and expression of the murine Wilms' tumor gene (WT1) during kidney development
RL Mol. Biol. Cell 11:1707-1712 (1991).
RN [15]; RE0006722.
RX PUBMED: 8170946.
RA Varanasi R., Bardeesy N., Ghahremani M., Petruzzi M.J., Nowak N., Adam M. A., Grundy P., Shows T. B., Pelletier J.
RT Fine structure analysis of the WT1 gene in sporadic Wilms tumors
RL Proc. Natl. Acad. Sci. USA 91:3554-3558 (1994).
RN [16]; RE0006726.
RX PUBMED: 7657166.
RA Maheswaran S., Englert C., Bennett P., Heinrich G., Haber D. A.
RT The WT1 gene product stabilizes p53 and inhibits p53-mediated apoptosis
RL Genes Dev. 9:2143-2156 (1995).
RN [17]; RE0006727.
RX PUBMED: 7588596.
RA Englert C., Hou X., Maheswaran S., Bennett P., Ngwu C., Re G. G., Garvin A. J., Rosner M. R., Haber D. A.
RT WT1 suppresses synthesis of the epidermal growth factor receptor and induces apoptosis
RL EMBO J. 14:4662-4676 (1995).
RN [18]; RE0013915.
RX PUBMED: 1338906.
RA Baird P. N., Santos A., Groves N., Jadresic L., Cowell J. K.
RT Constitutional mutations in the WT1 gene in patients with Denys-Drash syndrome
RL Hum. Mol. Genet. 1:301-305 (1992).
RN [19]; RE0013921.
RX PUBMED: 9529364.
RA Jeanpierre C., Denamur E., Henry I., Cabanis M. O., Luce S., Cecille A., Elion J., Peuchmaur M., Loirat C., Niaudet P., Gubler M. C., Junien C.
RT 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
RL Am. J. Hum. Genet. 62:824-833 (1998).
RN [20]; RE0013924.
RX PUBMED: 8388765.
RA Little M. H., Williamson K. A., Mannens M., Kelsey A., Gosden C., Hastie N. D., van Heyningen V.
RT Evidence that WT1 mutations in Denys-Drash syndrome patients may act in a dominant-negative fashion
RL Hum. Mol. Genet. 2:259-264 (1993).
RN [21]; RE0013926.
RX PUBMED: 1655284.
RA Pelletier J., Bruening W., Kashtan C. E., Mauer S. M., Manivel J. C., Striegel J. E., Houghton D. C., Junien C., Habib R., Fouser L., Fine R. N., Silverman B. L., Haber D. A., Housman D.
RT Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome
RL Cell 67:437-447 (1991).
RN [22]; RE0013960.
RX PUBMED: 8401592.
RA Park S., Schalling M., Bernard A., Maheswaran S., Shipley G. C., Roberts D., Fletcher J., Shipman R., Rheinwald J., Demetri G.
RT The Wilms tumour gene WT1 is expressed in murine mesoderm-derived tissues and mutated in a human mesothelioma
RL Nat. Genet. 4:415-420 (1993).
RN [23]; RE0013961.
RX PUBMED: 9108089.
RA Schumacher V., Schneider S., Figge A., Wildhardt G., Harms D., Schmidt D., Weirich A., Ludwig R., Royker-Pokora B.
RT Correlation of germ-line mutations and two-hit inactivation of the WT1 gene with Wilms tumors of stromal-predominant histology
RL Proc. Natl. Acad. Sci. USA 94:3972-3977 (1997).
RN [24]; RE0021966.
RX PUBMED: 1313285.
RA HABER D.A., BUCKLER A.J.
RT WT1: a novel tumor suppressor gene inactivated in Wilms' tumor.
RL New Biol. 4:97-106 (1992).
RN [25]; RE0021967.
RX PUBMED: 1658787.
RA HABER D.A., SOHN R.L., BUCKLER A.J., PELLETIER J., CALL K.M., HOUSMAN D.E.
RT Alternative splicing and genomic structure of the Wilms tumor gene WT1.
RL Proc. Natl. Acad. Sci. USA 88:9618-9622 (1991).
RN [26]; RE0021968.
RX PUBMED: 2154702.
RA GESSLER M., POUSTKA A., CAVENEE W., NEVE R.L., ORKIN S.H., BRUNS G.A.P.
RT Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping.
RL Nature 343:774-778 (1990).
RN [27]; RE0049501.
RX PUBMED: 16087727.
RA Reizner N., Maor S., Sarfstein R., Abramovitch S., Welshons W. V., Curran E. M., Lee A. V., Werner H.
RT The WT1 Wilms' tumor suppressor gene product interacts with estrogen receptor-alpha and regulates IGF-I receptor gene transcription in breast cancer cells.
RL J. Mol. Endocrinol. 35:135-144 (2005).
RN [28]; RE0020468.
RX PUBMED: 8943350.
RA Johnstone R. W., See R. H., Sells S. F., Wang J., Muthukkumar S., Englert C., Haber D. A., Licht J. D., Sugrue S. P., Roberts T., Rangnekar V. M., Shi Y.
RT A novel repressor, par-4, modulates transcription and growth suppression functions of the Wilms tumor suppressor WT1
RL Mol. Cell. Biol. 16:6945-6956 (1996).
XX
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