CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-004786
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Wilms tumor protein 
Protein Synonyms/Alias
 WT33 
Gene Name
 WT1 
Gene Synonyms/Alias
  
Created Date
 July 27, 2013 
Organism
 Homo sapiens (Human) 
NCBI Taxa ID
 9606 
Lysine Modification
Position
Peptide
Type
References
73PPPHSFIKQEPSWGGsumoylation[1]
177QFPNHSFKHEDPMGQsumoylation[1]
Reference
 [1] SUMO-1 modification of the Wilms' tumor suppressor WT1.
 Smolen GA, Vassileva MT, Wells J, Matunis MJ, Haber DA.
 Cancer Res. 2004 Nov 1;64(21):7846-51. [PMID: 15520190] 
Functional Description
 Transcription factor that plays an important role in cellular development and cell survival. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may act as transcription factors. Isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing. Isoform 1 has lower affinity for DNA, and can bind RNA. 
Sequence Annotation
 ZN_FING 323 347 C2H2-type 1.
 ZN_FING 353 377 C2H2-type 2.
 ZN_FING 383 405 C2H2-type 3.
 ZN_FING 414 438 C2H2-type 4.
 REGION 367 381 Important for interaction with target
 REGION 393 401 Important for interaction with target
 MOTIF 408 410 KTS motif.
 CROSSLNK 73 73 Glycyl lysine isopeptide (Lys-Gly)
 CROSSLNK 177 177 Glycyl lysine isopeptide (Lys-Gly)  
Keyword
 3D-structure; Alternative initiation; Alternative splicing; Chromosomal rearrangement; Complete proteome; Cytoplasm; Disease mutation; DNA-binding; Isopeptide bond; Metal-binding; Nucleus; Reference proteome; Repeat; RNA editing; RNA-binding; Transcription; Transcription regulation; Tumor suppressor; Ubl conjugation; Zinc; Zinc-finger. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 449 AA 
Protein Sequence
MGSDVRDLNA LLPAVPSLGG GGGCALPVSG AAQWAPVLDF APPGASAYGS LGGPAPPPAP 60
PPPPPPPPHS FIKQEPSWGG AEPHEEQCLS AFTVHFSGQF TGTAGACRYG PFGPPPPSQA 120
SSGQARMFPN APYLPSCLES QPAIRNQGYS TVTFDGTPSY GHTPSHHAAQ FPNHSFKHED 180
PMGQQGSLGE QQYSVPPPVY GCHTPTDSCT GSQALLLRTP YSSDNLYQMT SQLECMTWNQ 240
MNLGATLKGV AAGSSSSVKW TEGQSNHSTG YESDNHTTPI LCGAQYRIHT HGVFRGIQDV 300
RRVPGVAPTL VRSASETSEK RPFMCAYPGC NKRYFKLSHL QMHSRKHTGE KPYQCDFKDC 360
ERRFSRSDQL KRHQRRHTGV KPFQCKTCQR KFSRSDHLKT HTRTHTGKTS EKPFSCRWPS 420
CQKKFARSDE LVRHHNMHQR NMTKLQLAL 449 
Gene Ontology
 GO:0005737; C:cytoplasm; ISS:UniProtKB.
 GO:0016607; C:nuclear speck; IDA:UniProtKB.
 GO:0005730; C:nucleolus; IEA:UniProtKB-SubCell.
 GO:0005654; C:nucleoplasm; IDA:UniProtKB.
 GO:0003723; F:RNA binding; IEA:UniProtKB-KW.
 GO:0001077; F:RNA polymerase II core promoter proximal region sequence-specific DNA binding transcription factor activity involved in positive regulation of transcription; ISS:UniProtKB.
 GO:0043565; F:sequence-specific DNA binding; IDA:UniProtKB.
 GO:0044212; F:transcription regulatory region DNA binding; IDA:UniProtKB.
 GO:0008270; F:zinc ion binding; IDA:UniProtKB.
 GO:0035802; P:adrenal cortex formation; ISS:UniProtKB.
 GO:0006309; P:apoptotic DNA fragmentation; IDA:UniProtKB.
 GO:0001658; P:branching involved in ureteric bud morphogenesis; IGI:UniProtKB.
 GO:0043010; P:camera-type eye development; ISS:UniProtKB.
 GO:0060923; P:cardiac muscle cell fate commitment; ISS:BHF-UCL.
 GO:0071320; P:cellular response to cAMP; IEP:UniProtKB.
 GO:0071371; P:cellular response to gonadotropin stimulus; IDA:UniProtKB.
 GO:0060539; P:diaphragm development; ISS:UniProtKB.
 GO:0007281; P:germ cell development; ISS:UniProtKB.
 GO:0032836; P:glomerular basement membrane development; IMP:UniProtKB.
 GO:0072112; P:glomerular visceral epithelial cell differentiation; ISS:UniProtKB.
 GO:0030539; P:male genitalia development; ISS:UniProtKB.
 GO:0008584; P:male gonad development; IEP:UniProtKB.
 GO:0060231; P:mesenchymal to epithelial transition; ISS:UniProtKB.
 GO:0072207; P:metanephric epithelium development; IEP:UniProtKB.
 GO:0072075; P:metanephric mesenchyme development; ISS:UniProtKB.
 GO:0072284; P:metanephric S-shaped body morphogenesis; IGI:UniProtKB.
 GO:0043066; P:negative regulation of apoptotic process; IGI:UniProtKB.
 GO:0030308; P:negative regulation of cell growth; IDA:UniProtKB.
 GO:2000195; P:negative regulation of female gonad development; ISS:UniProtKB.
 GO:0072302; P:negative regulation of metanephric glomerular mesangial cell proliferation; ISS:UniProtKB.
 GO:0000122; P:negative regulation of transcription from RNA polymerase II promoter; IDA:MGI.
 GO:0045892; P:negative regulation of transcription, DNA-dependent; IDA:UniProtKB.
 GO:0017148; P:negative regulation of translation; IDA:UniProtKB.
 GO:0060421; P:positive regulation of heart growth; ISS:UniProtKB.
 GO:2000020; P:positive regulation of male gonad development; ISS:UniProtKB.
 GO:2001076; P:positive regulation of metanephric ureteric bud development; ISS:UniProtKB.
 GO:0045893; P:positive regulation of transcription, DNA-dependent; IDA:UniProtKB.
 GO:0072166; P:posterior mesonephric tubule development; ISS:UniProtKB.
 GO:0003156; P:regulation of organ formation; ISS:UniProtKB.
 GO:0008380; P:RNA splicing; ISS:UniProtKB.
 GO:0007530; P:sex determination; IDA:UniProtKB.
 GO:0007356; P:thorax and anterior abdomen determination; ISS:UniProtKB.
 GO:0001570; P:vasculogenesis; ISS:UniProtKB.
 GO:0061032; P:visceral serous pericardium development; IGI:UniProtKB. 
Interpro
 IPR017987; Wilms_tumour.
 IPR000976; Wilms_tumour_N.
 IPR007087; Znf_C2H2.
 IPR015880; Znf_C2H2-like.
 IPR013087; Znf_C2H2/integrase_DNA-bd. 
Pfam
 PF02165; WT1 
SMART
 SM00355; ZnF_C2H2 
PROSITE
 PS00028; ZINC_FINGER_C2H2_1
 PS50157; ZINC_FINGER_C2H2_2 
PRINTS
 PR00049; WILMSTUMOUR.