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PR00049

Identifier
WILMSTUMOUR  [View Relations]  [View Alignment]  
Accession
PR00049
No. of Motifs
4
Creation Date
05-APR-1993  (UPDATE 17-JUN-1999)
Title
Wilm's tumour protein signature
Database References

INTERPRO; IPR000976
Literature References
1. GESSLER, M., POUSTKA, A., CAVENEE, W., NEVE, R.L., ORKIN, S.H. AND
BRUNS, G.A.P.
Homozygous  deletion in Wilms-tumors of a zinc-finger gene identified by
chromosome jumping. 
NATURE 343 774-778 (1990).
 
2. LITTLE, M.H., PROSSER, J., CONDIE, A., SMITH, P.J., VAN HEYNINGEN, V.,
AND HASTIE, N.D. 
Zinc finger point mutations within the WT1 gene in Wilms-tumor patients.
PROC.NATL.ACAD.SCI.U.S.A. 89(11) 4791-4795 (1992).
 
3. RAUSCHER, F.J.
The WT1 Wilms-tumor gene-product - A developmentally-regulated
transcription factor in the kidney that functions as a tumor-suppressor.
FASEB J. 7(10) 896-903 (1993).
 
4. BUCKLER, A.J., PELLETIER, J., HABER, D.A., GLASER, T. AND HOUSMAN, D.E.
Isolation, characterisation and expression of the murine Wilm's-tumor gene
MOL.CELL.BIOL. 11(3) 1707-1712 (1991). 

Documentation
Wilm's tumour (WT) is an embryonal malignancy of the kidney, affecting
around 1 in 10,000 infants. It occurs in both sporadic and hereditary
forms. Inactivation of WT1 is one of the causes of Wilm's tumour. Defects
in the WT1 gene are also associated with Denys-Drash Syndrome (DDS), which
is characterised by typical nephropathy and genital abnormalities. The
WT1 gene product shows similarity to the zinc fingers of the mammalian
growth regulated EGR1 and EGR2 proteins [1-4].
 
WILMSTUMOUR is a 4-element fingerprint that provides a signature for Wilm's
tumour proteins. The fingerprint was derived from an initial alignment
of 2 sequences: the motifs were drawn from well-conserved, N-terminal
Gly/Pro-rich regions, motifs 2-4 spanning the inhibitory domain. Two
iterations on OWL22.1 were required to reach convergence, at which point
a true set comprising 5 sequences was identified.
 
An update on SPTR37_9f identified a true set of 5 sequences, and 1
partial match.
Summary Information
   5 codes involving  4 elements
0 codes involving 3 elements
1 codes involving 2 elements
Composite Feature Index
45555
30000
21100
1234
True Positives
Q15881        WT1_HUMAN     WT1_MOUSE     WT1_PIG       
WT1_RAT
True Positive Partials
Codes involving 2 elements
O42223
Sequence Titles
Q15881      WILMS TUMOR GENE 1, EXON 1 - HOMO SAPIENS (HUMAN). 
WT1_HUMAN WILMS' TUMOR PROTEIN (WT33) - HOMO SAPIENS (HUMAN).
WT1_MOUSE WILMS' TUMOR PROTEIN HOMOLOG - MUS MUSCULUS (MOUSE).
WT1_PIG WILMS' TUMOR PROTEIN HOMOLOG - SUS SCROFA (PIG).
WT1_RAT WILMS' TUMOR PROTEIN HOMOLOG - RATTUS NORVEGICUS (RAT).

O42223 WILMS TUMOR 1 PROTEIN - TRACHEMYS SCRIPTA (RED-EARED SLIDER TURTLE).
Scan History
OWL22_1    2  50   NSINGLE    
OWL26_0 1 100 NSINGLE
SPTR37_9f 2 100 NSINGLE
Initial Motifs
Motif 1  width=17
Element Seqn Id St Int Rpt
MGSDVRDLNALLPAVPS WT1_HUMAN 1 1 -
MGSDVRDLNALLPAVSS WT1_MOUSE 1 1 -

Motif 2 width=17
Element Seqn Id St Int Rpt
GGGGGCALPVSGAAQWA WT1_HUMAN 19 1 -
GGGGGCGLPVSGARQWA WT1_MOUSE 20 2 -

Motif 3 width=15
Element Seqn Id St Int Rpt
VLDFAPPGASAYGSL WT1_HUMAN 37 1 -
VLDFAPPGASAYGSL WT1_MOUSE 38 1 -

Motif 4 width=15
Element Seqn Id St Int Rpt
GGPAPPPAPPPPPPP WT1_HUMAN 52 0 -
GGPAPPPAPPPPPPP WT1_MOUSE 53 0 -
Final Motifs
Motif 1  width=17
Element Seqn Id St Int Rpt
MGSDVRDLNALLPAVPS Q15881 1 1 -
MGSDVRDLNALLPAVPS WT1_HUMAN 1 1 -
MGSDVRDLNALLPAVSS WT1_MOUSE 1 1 -
MGSDVRDLNALLPAVSS WT1_RAT 1 1 -
MGSDVRDLNALLPAVPS WT1_PIG 1 1 -

Motif 2 width=17
Element Seqn Id St Int Rpt
GGGGGCALPVSGAAQWA Q15881 19 1 -
GGGGGCALPVSGAAQWA WT1_HUMAN 19 1 -
GGGGGCGLPVSGARQWA WT1_MOUSE 20 2 -
GGGGGCGLPVSGARQWA WT1_RAT 19 1 -
GGGGGCALPVSGAAEWA WT1_PIG 19 1 -

Motif 3 width=15
Element Seqn Id St Int Rpt
VLDFAPPGASAYGSL Q15881 37 1 -
VLDFAPPGASAYGSL WT1_HUMAN 37 1 -
VLDFAPPGASAYGSL WT1_MOUSE 38 1 -
VLDFAPPGASAYGSL WT1_RAT 37 1 -
VLDFAPPGASAYGSL WT1_PIG 37 1 -

Motif 4 width=15
Element Seqn Id St Int Rpt
GGPAPPPAPPPPPPP Q15881 52 0 -
GGPAPPPAPPPPPPP WT1_HUMAN 52 0 -
GGPAPPPAPPPPPPP WT1_MOUSE 53 0 -
GGPAPPPAPPPPPPP WT1_RAT 52 0 -
GGPAPPPAPPPPPPP WT1_PIG 52 0 -