Summary: This gene encodes a transcription factor that contains four zinc-finger motifs at the C-terminus and a proline/glutamine-rich DNA-binding domain at the N-terminus. It has an essential role in the normal development of the urogenital system, and it is mutated in a small subset of patients with Wilms tumor. This gene exhibits complex tissue-specific and polymorphic imprinting pattern, with biallelic, and monoallelic expression from the maternal and paternal alleles in different tissues. Multiple transcript variants have been described. In several variants, there is evidence for the use of a non-AUG (CUG) translation initiation codon upstream of, and in-frame with the first AUG. Authors of PMID:7926762 also provide evidence that WT1 mRNA undergoes RNA editing in human and rat, and that this process is tissue-restricted and developmentally regulated. [provided by RefSeq, Mar 2015].

Wilms tumor 1MIM:607102Ensembl:ENSG00000184937HGNC:HGNC:12796PA3739511p13

GO terms in WT1

Term TypeEvidence TypeGO Term IDGO Des.
MFIBAGO:0000981RNA polymerase II transcription factor activity, sequence-specific DNA binding
MFISAGO:0000981RNA polymerase II transcription factor activity, sequence-specific DNA binding
MFISMGO:0000981RNA polymerase II transcription factor activity, sequence-specific DNA binding
MFNASGO:0000981RNA polymerase II transcription factor activity, sequence-specific DNA binding
MFISSGO:0001077transcriptional activator activity, RNA polymerase II proximal promoter sequence-specific DNA binding
MFISSGO:0003700DNA-binding transcription factor activity
MFNASGO:0003700DNA-binding transcription factor activity
MFIEAGO:0003723RNA binding
MFIPIGO:0005515protein binding
MFIDAGO:0008270zinc ion binding
MFIDAGO:0010385double-stranded methylated DNA binding
MFIDAGO:0043565sequence-specific DNA binding
MFIDAGO:0044212transcription regulatory region DNA binding
MFIDAGO:0044729hemi-methylated DNA-binding
MFIPIGO:0070742C2H2 zinc finger domain binding
CCIDAGO:0016607nuclear speck
BPIDAGO:0000122negative regulation of transcription by RNA polymerase II
BPISSGO:0001657ureteric bud development
BPIGIGO:0001658branching involved in ureteric bud morphogenesis
BPIGIGO:0001822kidney development
BPISSGO:0003156regulation of animal organ formation
BPISSGO:0006355regulation of transcription, DNA-templated
BPNASGO:0006355regulation of transcription, DNA-templated
BPIBAGO:0006357regulation of transcription by RNA polymerase II
BPISSGO:0006357regulation of transcription by RNA polymerase II
BPIEAGO:0006366transcription by RNA polymerase II
BPISSGO:0007281germ cell development
BPISSGO:0007356thorax and anterior abdomen determination
BPIGIGO:0007507heart development
BPIDAGO:0007530sex determination
BPIBAGO:0008285negative regulation of cell proliferation
BPIDAGO:0008285negative regulation of cell proliferation
BPISSGO:0008380RNA splicing
BPISSGO:0008406gonad development
BPIEPGO:0008584male gonad development
BPISSGO:0009888tissue development
BPIDAGO:0030308negative regulation of cell growth
BPIGIGO:0030325adrenal gland development
BPISSGO:0030539male genitalia development
BPISSGO:0030855epithelial cell differentiation
BPIGIGO:0032835glomerulus development
BPIMPGO:0032836glomerular basement membrane development
BPISSGO:0035802adrenal cortex formation
BPISSGO:0043010camera-type eye development
BPIBAGO:0043066negative regulation of apoptotic process
BPIGIGO:0043066negative regulation of apoptotic process
BPIDAGO:0045892negative regulation of transcription, DNA-templated
BPIDAGO:0045893positive regulation of transcription, DNA-templated
BPIGIGO:0045893positive regulation of transcription, DNA-templated
BPIEAGO:0045944positive regulation of transcription by RNA polymerase II
BPISSGO:0060231mesenchymal to epithelial transition
BPISSGO:0060421positive regulation of heart growth
BPISSGO:0060539diaphragm development
BPISSGO:0060923cardiac muscle cell fate commitment
BPIGIGO:0061032visceral serous pericardium development
BPIBAGO:0071320cellular response to cAMP
BPIEPGO:0071320cellular response to cAMP
BPIBAGO:0071371cellular response to gonadotropin stimulus
BPIDAGO:0071371cellular response to gonadotropin stimulus
BPISSGO:0072075metanephric mesenchyme development
BPIBAGO:0072112glomerular visceral epithelial cell differentiation
BPISSGO:0072112glomerular visceral epithelial cell differentiation
BPISSGO:0072166posterior mesonephric tubule development
BPIEPGO:0072207metanephric epithelium development
BPIGIGO:0072284metanephric S-shaped body morphogenesis
BPISSGO:0072302negative regulation of metanephric glomerular mesangial cell proliferation
BPISSGO:2000020positive regulation of male gonad development
BPISSGO:2000195negative regulation of female gonad development
BPISSGO:2001076positive regulation of metanephric ureteric bud development

Gene expression in normal tissue: WT1

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in WT1

DatabasePathway IDPathway Des.
biocartatertpathwayoverview of telomerase protein component gene htert transcriptional regulation
biocartatidpathwaychaperones modulate interferon signaling pathway
pidp73pathwayp73 transcription factor network
pidtelomerasepathwayRegulation of Telomerase
kegghsa05202Transcriptional misregulation in cancer - Homo sapiens (human)
wikipathwaysWP2572Primary Focal Segmental Glomerulosclerosis FSGS

Gene-Drug: Aster Plot

Drug IDDrug NameModel Num.

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in WT1