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Protein

Wilms tumor protein homolog

Gene

WT1

Organism
Sus scrofa (Pig)
Status
Reviewed-Annotation score: -Experimental evidence at transcript leveli

Functioni

Transcription factor that plays an important role in cellular development and cell survival. Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3'. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. 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.By similarity

Miscellaneous

Presence of the KTS motif hinders interactions between DNA and zinc-finger 4.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei424Important for interaction with target DNABy similarity1
Sitei430Important for interaction with target DNABy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri323 – 347C2H2-type 1PROSITE-ProRule annotationAdd BLAST25
Zinc fingeri353 – 377C2H2-type 2PROSITE-ProRule annotationAdd BLAST25
Zinc fingeri383 – 405C2H2-type 3PROSITE-ProRule annotationAdd BLAST23
Zinc fingeri414 – 438C2H2-type 4PROSITE-ProRule annotationAdd BLAST25

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionDNA-binding, RNA-binding
Biological processTranscription, Transcription regulation
LigandMetal-binding, Zinc

Names & Taxonomyi

Protein namesi
Recommended name:
Wilms tumor protein homolog
Gene namesi
Name:WT1
OrganismiSus scrofa (Pig)
Taxonomic identifieri9823 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaSuinaSuidaeSus
Proteomesi
  • UP000008227 Componenti: Unplaced

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Nucleus

Pathology & Biotechi

Keywords - Diseasei

Tumor suppressor

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000471331 – 449Wilms tumor protein homologAdd BLAST449

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki73Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity
Cross-linki177Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity
Cross-linki444Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)By similarity

Keywords - PTMi

Isopeptide bond, Ubl conjugation

Proteomic databases

PaxDbiO62651
PeptideAtlasiO62651
PRIDEiO62651

Expressioni

Developmental stagei

Expressed during kidney development.

Interactioni

Subunit structurei

Interacts with ZNF224 via the zinc-finger region. Interacts with WTAP, AMER1 and SRY. Homodimer. Interacts with WTIP. Interacts with actively translating polysomes. Detected in nuclear ribonucleoprotein (mRNP) particles. Interacts with U2AF2. Interacts with HNRNPU via the zinc-finger region. Interacts with CITED2. Interacts with RBM4 (By similarity).By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi9823.ENSSSCP00000019417

Structurei

3D structure databases

ProteinModelPortaliO62651
SMRiO62651
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni367 – 381Important for interaction with target DNABy similarityAdd BLAST15
Regioni393 – 401Important for interaction with target DNABy similarity9

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi408 – 410KTS motifBy similarity3

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi28 – 83Pro-richAdd BLAST56

Domaini

Binds to DNA motifs with the sequence 5'-GCG(T/G)GGGCG-3' via its C2H2-type zinc fingers. Starting from the N-terminus, the second zinc finger binds to the 3'-GCG motif, the middle zinc finger interacts with the central TGG motif, and the C-terminal zinc finger binds to the 5'-GCG motif. Binds double-stranded target DNA, irrespective of the cytosine methylation status. Has reduced affinity for target DNA where the cytosines have been oxidized to 5-hydroxymethylcytosine, 5-formylcytosine or 5-carboxylcytosine.By similarity

Sequence similaritiesi

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri323 – 347C2H2-type 1PROSITE-ProRule annotationAdd BLAST25
Zinc fingeri353 – 377C2H2-type 2PROSITE-ProRule annotationAdd BLAST25
Zinc fingeri383 – 405C2H2-type 3PROSITE-ProRule annotationAdd BLAST23
Zinc fingeri414 – 438C2H2-type 4PROSITE-ProRule annotationAdd BLAST25

Keywords - Domaini

Repeat, Zinc-finger

Phylogenomic databases

eggNOGiKOG1721 Eukaryota
COG5048 LUCA
HOVERGENiHBG006960
InParanoidiO62651
KOiK09234

Family and domain databases

InterProiView protein in InterPro
IPR017987 Wilms_tumour
IPR000976 Wilms_tumour_N
IPR036236 Znf_C2H2_sf
IPR013087 Znf_C2H2_type
PANTHERiPTHR44601 PTHR44601, 1 hit
PfamiView protein in Pfam
PF02165 WT1, 1 hit
SMARTiView protein in SMART
SM00355 ZnF_C2H2, 4 hits
SUPFAMiSSF57667 SSF57667, 2 hits
PROSITEiView protein in PROSITE
PS00028 ZINC_FINGER_C2H2_1, 4 hits
PS50157 ZINC_FINGER_C2H2_2, 4 hits

Sequences (4)i

Sequence statusi: Complete.

This entry describes 4 isoformsi produced by alternative splicing. AlignAdd to basket

Note: Experimental confirmation may be lacking for some isoforms.
Isoform 1 (identifier: O62651-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MGSDVRDLNA LLPAVPSLGG GGGCALPVSG AAEWAPVLDF APPGASAYGS
60 70 80 90 100
LGGPAPPPAP PPPPPPPPHS FIKQEPSWGG AEPHEEQCLS AFTVHFSGQF
110 120 130 140 150
TGTAGACRYE PFGPPPPSQA SSGQARMFPN APYLPSCLES QPAIRNQGYS
160 170 180 190 200
TVTFDGTPSY GHTPSHHAAQ FPNHSFKHED PMGQQGSLGE QQYSVPPPVY
210 220 230 240 250
GCHTSTDSCT GSQALLLRTP YSSDNLYQMT SQLECMTWNQ MNLGATLKGV
260 270 280 290 300
AAGTSSSMKW TEGQSNHGAG YESDSHATPI LCGAQYRIHT HGVFRGIQDV
310 320 330 340 350
RRVPGVAPTL VRSASETSEK RPFMCAYPGC NKRYFKLSHL QMHSRKHTGE
360 370 380 390 400
KPYQCDFKDC ERRFSRSDQL KRHQRRHTGV KPFQCKTCQR KFSRSDHLKT
410 420 430 440
HTRTHTGKTS EKPFSCRWPS CQKKFARSDE LVRHHNMHQR NMSKLQLAL
Length:449
Mass (Da):49,166
Last modified:August 1, 1998 - v1
Checksum:i9C3E557B96F5A7B3
GO
Isoform 2 (identifier: O62651-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     250-266: Missing.
     408-410: Missing.

Show »
Length:429
Mass (Da):47,127
Checksum:iBB1FC9A497B09469
GO
Isoform 3 (identifier: O62651-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     250-266: Missing.

Show »
Length:432
Mass (Da):47,443
Checksum:i891551FAB065275F
GO
Isoform 4 (identifier: O62651-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     408-410: Missing.

Show »
Length:446
Mass (Da):48,850
Checksum:iAA3959247BAA0A56
GO

RNA editingi

Edited at position 281.By similarity
Partially edited.By similarity

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural varianti281L → P in RNA edited version. 1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_006870250 – 266Missing in isoform 2 and isoform 3. 1 PublicationAdd BLAST17
Alternative sequenceiVSP_006871408 – 410Missing in isoform 2 and isoform 4. 1 Publication3

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB010969 mRNA Translation: BAA28147.1
RefSeqiNP_001001264.1, NM_001001264.1 [O62651-1]
UniGeneiSsc.20977

Genome annotation databases

GeneIDi397338
KEGGissc:397338

Keywords - Coding sequence diversityi

Alternative splicing, RNA editing

Similar proteinsi

Entry informationi

Entry nameiWT1_PIG
AccessioniPrimary (citable) accession number: O62651
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 15, 1998
Last sequence update: August 1, 1998
Last modified: May 23, 2018
This is version 120 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families

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