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Wilms tumor protein homolog

 WT1_RAT                 Reviewed;         448 AA.
P49952;
01-OCT-1996, integrated into UniProtKB/Swiss-Prot.
01-OCT-1996, sequence version 1.
13-FEB-2019, entry version 142.
RecName: Full=Wilms tumor protein homolog;
Name=Wt1; Synonyms=Wt-1;
Rattus norvegicus (Rat).
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha;
Muroidea; Muridae; Murinae; Rattus.
NCBI_TaxID=10116;
[1]
NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS 1; 2; 3 AND 4).
STRAIN=Sprague-Dawley; TISSUE=Kidney;
PubMed=1330293;
Sharma P.M., Yang X., Bowman M., Roberts V., Sukumar S.;
"Molecular cloning of rat Wilms' tumor complementary DNA and a study
of messenger RNA expression in the urogenital system and the brain.";
Cancer Res. 52:6407-6412(1992).
[2]
RNA EDITING OF POSITION 280.
PubMed=7926762; DOI=10.1101/gad.8.6.720;
Sharma P.M., Bowman M., Madden S.L., Rauscher F.J. III, Sukumar S.;
"RNA editing in the Wilms' tumor susceptibility gene, WT1.";
Genes Dev. 8:720-731(1994).
-!- FUNCTION: 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.
{ECO:0000250|UniProtKB:P19544}.
-!- SUBUNIT: 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 (By similarity). {ECO:0000250}.
-!- SUBCELLULAR LOCATION: Isoform 1: Nucleus speckle {ECO:0000250}.
-!- SUBCELLULAR LOCATION: Isoform 4: Nucleus, nucleoplasm
{ECO:0000250}.
-!- SUBCELLULAR LOCATION: Nucleus {ECO:0000250}. Nucleus, nucleolus
{ECO:0000250}. Cytoplasm {ECO:0000250}. Nucleus speckle
{ECO:0000250}. Note=Shuttles between nucleus and cytoplasm.
{ECO:0000250}.
-!- ALTERNATIVE PRODUCTS:
Event=Alternative splicing; Named isoforms=4;
Name=1;
IsoId=P49952-1; Sequence=Displayed;
Name=2;
IsoId=P49952-2; Sequence=VSP_006872, VSP_006873;
Name=3;
IsoId=P49952-3; Sequence=VSP_006872;
Name=4;
IsoId=P49952-4; Sequence=VSP_006873;
-!- TISSUE SPECIFICITY: Kidney.
-!- DEVELOPMENTAL STAGE: Expressed during kidney development.
-!- DOMAIN: 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. {ECO:0000250|UniProtKB:P19544}.
-!- RNA EDITING: Modified_positions=280 {ECO:0000269|PubMed:7926762};
Note=Partially edited.;
-!- MISCELLANEOUS: Presence of the KTS motif hinders interactions
between DNA and zinc-finger 4. {ECO:0000250}.
-!- SIMILARITY: Belongs to the EGR C2H2-type zinc-finger protein
family. {ECO:0000305}.
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EMBL; X69716; CAA49373.1; -; mRNA.
PIR; S33926; S33926.
RefSeq; NP_113722.2; NM_031534.2.
UniGene; Rn.92531; -.
ProteinModelPortal; P49952; -.
SMR; P49952; -.
BioGrid; 246993; 1.
STRING; 10116.ENSRNOP00000060038; -.
PaxDb; P49952; -.
PRIDE; P49952; -.
GeneID; 24883; -.
KEGG; rno:24883; -.
UCSC; RGD:3974; rat. [P49952-1]
CTD; 7490; -.
RGD; 3974; Wt1.
eggNOG; KOG1721; Eukaryota.
eggNOG; COG5048; LUCA.
HOGENOM; HOG000230937; -.
HOVERGEN; HBG006960; -.
InParanoid; P49952; -.
KO; K09234; -.
OrthoDB; 1318335at2759; -.
PhylomeDB; P49952; -.
PRO; PR:P49952; -.
Proteomes; UP000002494; Unplaced.
GO; GO:0005737; C:cytoplasm; ISS:UniProtKB.
GO; GO:0016607; C:nuclear speck; ISS:UniProtKB.
GO; GO:0005730; C:nucleolus; IEA:UniProtKB-SubCell.
GO; GO:0005654; C:nucleoplasm; ISS:UniProtKB.
GO; GO:0005634; C:nucleus; IDA:RGD.
GO; GO:0070742; F:C2H2 zinc finger domain binding; ISS:UniProtKB.
GO; GO:0001228; F:DNA-binding transcription activator activity, RNA polymerase II-specific; ISS:UniProtKB.
GO; GO:0003700; F:DNA-binding transcription factor activity; ISS:UniProtKB.
GO; GO:0000981; F:DNA-binding transcription factor activity, RNA polymerase II-specific; IBA:GO_Central.
GO; GO:0010385; F:double-stranded methylated DNA binding; ISS:UniProtKB.
GO; GO:0044729; F:hemi-methylated DNA-binding; ISS:UniProtKB.
GO; GO:0003723; F:RNA binding; IEA:UniProtKB-KW.
GO; GO:0043565; F:sequence-specific DNA binding; IDA:RGD.
GO; GO:0044212; F:transcription regulatory region DNA binding; ISS:UniProtKB.
GO; GO:0008270; F:zinc ion binding; ISS:UniProtKB.
GO; GO:0035802; P:adrenal cortex formation; ISS:UniProtKB.
GO; GO:0030325; P:adrenal gland development; ISS:UniProtKB.
GO; GO:0001658; P:branching involved in ureteric bud morphogenesis; ISS:UniProtKB.
GO; GO:0043010; P:camera-type eye development; ISS:UniProtKB.
GO; GO:0071320; P:cellular response to cAMP; IBA:GO_Central.
GO; GO:0071371; P:cellular response to gonadotropin stimulus; ISS:UniProtKB.
GO; GO:0060539; P:diaphragm development; ISS:UniProtKB.
GO; GO:0030855; P:epithelial cell differentiation; ISS:UniProtKB.
GO; GO:0007281; P:germ cell development; ISS:UniProtKB.
GO; GO:0032836; P:glomerular basement membrane development; ISS:UniProtKB.
GO; GO:0072112; P:glomerular visceral epithelial cell differentiation; ISS:UniProtKB.
GO; GO:0032835; P:glomerulus development; ISS:UniProtKB.
GO; GO:0008406; P:gonad development; ISS:UniProtKB.
GO; GO:0007507; P:heart development; ISS:UniProtKB.
GO; GO:0030539; P:male genitalia development; ISS:UniProtKB.
GO; GO:0060231; P:mesenchymal to epithelial transition; ISS:UniProtKB.
GO; GO:0072075; P:metanephric mesenchyme development; ISS:UniProtKB.
GO; GO:0072284; P:metanephric S-shaped body morphogenesis; ISS:UniProtKB.
GO; GO:0043066; P:negative regulation of apoptotic process; ISS:UniProtKB.
GO; GO:0030308; P:negative regulation of cell growth; ISS:UniProtKB.
GO; GO:0008285; P:negative regulation of cell population proliferation; ISS:UniProtKB.
GO; GO:2000195; P:negative regulation of female gonad development; ISS:UniProtKB.
GO; GO:0072302; P:negative regulation of metanephric glomerular mesangial cell proliferation; ISS:UniProtKB.
GO; GO:0045892; P:negative regulation of transcription, DNA-templated; ISS:UniProtKB.
GO; GO:0017148; P:negative regulation of translation; ISS:UniProtKB.
GO; GO:0043065; P:positive regulation of apoptotic process; ISS:UniProtKB.
GO; GO:0060421; P:positive regulation of heart growth; ISS:UniProtKB.
GO; GO:2000020; P:positive regulation of male gonad development; ISS:UniProtKB.
GO; GO:2001076; P:positive regulation of metanephric ureteric bud development; ISS:UniProtKB.
GO; GO:0045944; P:positive regulation of transcription by RNA polymerase II; IMP:RGD.
GO; GO:0045893; P:positive regulation of transcription, DNA-templated; IDA:UniProtKB.
GO; GO:0072166; P:posterior mesonephric tubule development; ISS:UniProtKB.
GO; GO:0003156; P:regulation of animal organ formation; ISS:UniProtKB.
GO; GO:0006357; P:regulation of transcription by RNA polymerase II; ISS:UniProtKB.
GO; GO:0006355; P:regulation of transcription, DNA-templated; ISS:UniProtKB.
GO; GO:0008380; P:RNA splicing; ISS:UniProtKB.
GO; GO:0007530; P:sex determination; ISS:UniProtKB.
GO; GO:0007356; P:thorax and anterior abdomen determination; ISS:UniProtKB.
GO; GO:0009888; P:tissue development; ISS:UniProtKB.
GO; GO:0001657; P:ureteric bud development; ISS:UniProtKB.
GO; GO:0001570; P:vasculogenesis; ISS:UniProtKB.
GO; GO:0061032; P:visceral serous pericardium development; ISS:UniProtKB.
InterPro; IPR017987; Wilms_tumour.
InterPro; IPR000976; Wilms_tumour_N.
InterPro; IPR036236; Znf_C2H2_sf.
InterPro; IPR013087; Znf_C2H2_type.
PANTHER; PTHR44601; PTHR44601; 1.
Pfam; PF02165; WT1; 1.
Pfam; PF00096; zf-C2H2; 2.
SMART; SM00355; ZnF_C2H2; 4.
SUPFAM; SSF57667; SSF57667; 2.
PROSITE; PS00028; ZINC_FINGER_C2H2_1; 4.
PROSITE; PS50157; ZINC_FINGER_C2H2_2; 4.
2: Evidence at transcript level;
Alternative splicing; Complete proteome; Cytoplasm; DNA-binding;
Isopeptide bond; Metal-binding; Nucleus; Reference proteome; Repeat;
RNA editing; RNA-binding; Transcription; Transcription regulation;
Tumor suppressor; Ubl conjugation; Zinc; Zinc-finger.
CHAIN 1 448 Wilms tumor protein homolog.
/FTId=PRO_0000047134.
ZN_FING 322 346 C2H2-type 1. {ECO:0000255|PROSITE-
ProRule:PRU00042}.
ZN_FING 352 376 C2H2-type 2. {ECO:0000255|PROSITE-
ProRule:PRU00042}.
ZN_FING 382 404 C2H2-type 3. {ECO:0000255|PROSITE-
ProRule:PRU00042}.
ZN_FING 413 437 C2H2-type 4. {ECO:0000255|PROSITE-
ProRule:PRU00042}.
REGION 366 380 Important for interaction with target
DNA. {ECO:0000250}.
REGION 392 400 Important for interaction with target
DNA. {ECO:0000250}.
MOTIF 407 409 KTS motif. {ECO:0000250}.
COMPBIAS 27 82 Pro-rich.
SITE 423 423 Important for interaction with target
DNA. {ECO:0000250}.
SITE 429 429 Important for interaction with target
DNA. {ECO:0000250}.
CROSSLNK 72 72 Glycyl lysine isopeptide (Lys-Gly)
(interchain with G-Cter in SUMO).
{ECO:0000250}.
CROSSLNK 176 176 Glycyl lysine isopeptide (Lys-Gly)
(interchain with G-Cter in SUMO).
{ECO:0000250}.
CROSSLNK 443 443 Glycyl lysine isopeptide (Lys-Gly)
(interchain with G-Cter in SUMO2).
{ECO:0000250|UniProtKB:P19544}.
VAR_SEQ 249 265 Missing (in isoform 2 and isoform 3).
{ECO:0000303|PubMed:1330293}.
/FTId=VSP_006872.
VAR_SEQ 407 409 Missing (in isoform 2 and isoform 4).
{ECO:0000303|PubMed:1330293}.
/FTId=VSP_006873.
VARIANT 280 280 L -> P (in RNA edited version).
SEQUENCE 448 AA; 49193 MW; 329AC9AC1FF73F76 CRC64;
MGSDVRDLNA LLPAVSSLGG GGGCGLPVSG ARQWAPVLDF APPGASAYGS LGGPAPPPAP
PPPPPPPHSF IKQEPSWGGA EPHEEQCLSA FTLHFSGQFT GTAGACRYGP FGPPPPSQAS
SGQARMFPNA PYLPSCLESQ PSIRNQGYST VTFDGAPSYG HTPSHHAAQF PNHSFKHEDP
MGQQGSLGEQ QYSVPPPVYG CHTPTDSCTG SQALLLRTPY SSDNLYQMTS QLECMTWNQM
NLGATLKGMA AGSSSSVKWT EGQSNHGTGY ESENHTTPIL CGAQYRIHTH GVFRGIQDVR
RVSGVAPTLV RSASETSEKR PFMCAYPGCN KRYFKLSHLQ MHSRKHTGEK PYQCDFKDCE
RRFSRSDQLK RHQRRHTGVK PFQCKTCQRK FSRSDHLKTH TRTHTGKTSE KPFSCRWHSC
QKKFARSDEL VRHHNMHQRN MTKLHVAL


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Kits Elisa; taq POLYMERASE

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Pathways :
WP1663: Homologous recombination
WP1676: Non-homologous end-joining
WP1036: Homologous recombination
WP1049: G Protein Signaling Pathways
WP1097: Non-homologous end joining
WP1153: Homologous recombination
WP1165: G Protein Signaling Pathways
WP1201: Non-homologous end joining
WP1205: Homologous recombination
WP1208: Non-homologous end joining
WP1216: Homologous recombination
WP1222: Non-homologous end joining
WP1234: Homologous recombination
WP1242: Non-homologous end joining
WP1258: Homologous recombination
WP1277: Non-homologous end joining
WP1296: Homologous recombination
WP1324: Non-homologous end joining
WP1362: Homologous recombination
WP1371: G Protein Signaling Pathways
WP1438: Influenza A virus infection
WP1493: Carbon assimilation C4 pathway
WP1502: Mitochondrial biogenesis
WP1531: Vitamin D synthesis
WP1566: Citrate cycle (TCA cycle)

Related Genes :
[Wt1 Wt-1] Wilms tumor protein homolog
[Wt1 Wt-1] Wilms tumor protein homolog
[WT1] Wilms tumor protein homolog
[wt1-a wt1] Wilms tumor protein homolog A (XWT1a) (xWT1)
[WT1] Wilms tumor protein homolog (Fragment)
[WT1] Wilms tumor protein homolog (Fragment)
[WTAP KIAA0105] Pre-mRNA-splicing regulator WTAP (Female-lethal(2)D homolog) (hFL(2)D) (WT1-associated protein) (Wilms tumor 1-associating protein)
[WT1] Wilms tumor protein (WT33)
[Wtap] Pre-mRNA-splicing regulator WTAP (Female-lethal(2)D homolog) (WT1-associated protein) (Wilms tumor 1-associating protein)
[wtip si:ch211-79l17.3] Wilms tumor protein 1-interacting protein homolog (WT1-interacting protein homolog)
[AMER1 FAM123B WTX] APC membrane recruitment protein 1 (Amer1) (Protein FAM123B) (Wilms tumor gene on the X chromosome protein)
[wt1-b wt1] Wilms tumor protein homolog B (XWT1b) (XeWT1)
[wt1] Wilms tumor protein homolog
[Wtip] Wilms tumor protein 1-interacting protein (WT1-interacting protein)
[Mtus1 Atbp Atip Cctsg1 Kiaa1288 Mtsg1] Microtubule-associated tumor suppressor 1 homolog (AT2 receptor-binding protein) (Angiotensin-II type 2 receptor-interacting protein) (Coiled-coiled tumor suppressor gene 1 protein) (Mitochondrial tumor suppressor 1 homolog)
[TWT1] Wilms tumor 1 protein
[WTIP] Wilms tumor protein 1-interacting protein (WT1-interacting protein)
[RPL10 DXS648E QM] 60S ribosomal protein L10 (Laminin receptor homolog) (Large ribosomal subunit protein uL16) (Protein QM) (Ribosomal protein L10) (Tumor suppressor QM)
[WT1 +KTS isoform] Wilms tumor 1 +KTS isoform
[WT1 -KTS isoform] Wilms tumor 1-KTS isoform
[LATS1 WARTS] Serine/threonine-protein kinase LATS1 (EC 2.7.11.1) (Large tumor suppressor homolog 1) (WARTS protein kinase) (h-warts)
[Lats2] Serine/threonine-protein kinase LATS2 (EC 2.7.11.1) (Kinase phosphorylated during mitosis protein) (Large tumor suppressor homolog 2) (Serine/threonine-protein kinase kpm)
[Litaf N4wbp3 Tbx1] Lipopolysaccharide-induced tumor necrosis factor-alpha factor homolog (LPS-induced TNF-alpha factor homolog) (Estrogen-enhanced transcript protein) (mEET) (LITAF-like protein) (NEDD4 WW domain-binding protein 3)
[LATS2 KPM] Serine/threonine-protein kinase LATS2 (EC 2.7.11.1) (Kinase phosphorylated during mitosis protein) (Large tumor suppressor homolog 2) (Serine/threonine-protein kinase kpm) (Warts-like kinase)
[TP63 KET P63 P73H P73L TP73L] Tumor protein 63 (p63) (Chronic ulcerative stomatitis protein) (CUSP) (Keratinocyte transcription factor KET) (Transformation-related protein 63) (TP63) (Tumor protein p73-like) (p73L) (p40) (p51)
[TP53 P53] Cellular tumor antigen p53 (Antigen NY-CO-13) (Phosphoprotein p53) (Tumor suppressor p53)
[CHEK2 CDS1 CHK2 RAD53] Serine/threonine-protein kinase Chk2 (EC 2.7.11.1) (CHK2 checkpoint homolog) (Cds1 homolog) (Hucds1) (hCds1) (Checkpoint kinase 2)
[Lats1 Warts] Serine/threonine-protein kinase LATS1 (EC 2.7.11.1) (Large tumor suppressor homolog 1) (WARTS protein kinase)
[L3MBTL1 KIAA0681 L3MBT L3MBTL] Lethal(3)malignant brain tumor-like protein 1 (H-l(3)mbt) (H-l(3)mbt protein) (L(3)mbt-like) (L(3)mbt protein homolog) (L3MBTL1)
[LTBR D12S370 TNFCR TNFR3 TNFRSF3] Tumor necrosis factor receptor superfamily member 3 (Lymphotoxin-beta receptor) (Tumor necrosis factor C receptor) (Tumor necrosis factor receptor 2-related protein) (Tumor necrosis factor receptor type III) (TNF-RIII) (TNFR-III)

Bibliography :
[29790264] Immune responses against tumour-associated antigen-derived cytotoxic T lymphocyte epitopes in cholangiocarcinoma patients.
[29357419] Modulation of apolipoprotein L1-microRNA-193a axis prevents podocyte dedifferentiation in high-glucose milieu.
[28668862] Clinicopathological Characteristics of Metaplastic Papillary Tumor of the Fallopian Tube.
[28529243] Over-Expression of miR-21 and Lower PTEN Levels in Wilms' Tumor with Aggressive Behavior.
[28301057] The correlation between LIN28B gene potentially functional variants and Wilms tumor susceptibility in Chinese children.
[27103452] RNA epigenetic modification: N6-methyladenosine.
[26848979] Aberrant epigenetic regulation in clear cell sarcoma of the kidney featuring distinct DNA hypermethylation and EZH2 overexpression.
[26729549] Gastrointestinal Findings in the Largest Series of Patients With Hereditary Biallelic Mismatch Repair Deficiency Syndrome: Report from the International Consortium.
[26655220] Wilms Tumor Suppressor, WT1, Cooperates with MicroRNA-26a and MicroRNA-101 to Suppress Translation of the Polycomb Protein, EZH2, in Mesenchymal Stem Cells.
[26155408] Trial watch: Naked and vectored DNA-based anticancer vaccines.