WT1 - WT1 transcription factor Gene
Also Known as GUD; AWT1; WAGR; WT-1; WT33; NPHS4; WIT-2
Species: Homo sapiens
About WT1
This gene has 22 transcripts (splice variants), 204 orthologues, 4 paralogues and is associated with 130 phenotypes. Biased expression in endometrium (RPKM 27.6), testis (RPKM 17.6) and 4 other tissues.
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]
WT1 Products (14)
| mRNA | Protein | Name |
|---|---|---|
| NM_000378.6 | NP_000369.4 | Wilms tumor protein isoform A |
| NM_001198551.2 | NP_001185480.1 | Wilms tumor protein isoform E |
| NM_001198552.2 | NP_001185481.1 | Wilms tumor protein isoform F |
| NM_001367854.1 | NP_001354783.1 | Wilms tumor protein isoform G |
| NM_001407044.1 | NP_001393973.1 | Wilms tumor protein isoform H |
| NM_001407045.1 | NP_001393974.1 | Wilms tumor protein isoform I |
| NM_001407046.1 | NP_001393975.1 | Wilms tumor protein isoform J |
| NM_001407047.1 | NP_001393976.1 | Wilms tumor protein isoform K |
| NM_001407048.1 | NP_001393977.1 | Wilms tumor protein isoform L |
| NM_001407049.1 | NP_001393978.1 | Wilms tumor protein isoform M |
| NM_001407050.1 | NP_001393979.1 | Wilms tumor protein isoform N |
| NM_001407051.1 | NP_001393980.1 | Wilms tumor protein isoform O |
| NM_024424.5 | NP_077742.3 | Wilms tumor protein isoform B |
| NM_024426.6 | NP_077744.4 | Wilms tumor protein isoform D |
| Molecular Function GO Annotation | Evidence | References | Source |
|---|---|---|---|
| enables C2H2 zinc finger domain binding |
IPI
IPI: Inferred from physical interaction
|
8119964 | GOA |
| enables DNA-binding transcription activator activity, RNA polymerase II-specific |
IDA
IDA: Inferred from direct assay
|
23042785 | GOA |
| enables RNA polymerase II cis-regulatory region sequence-specific DNA binding |
IDA
IDA: Inferred from direct assay
|
23042785 | GOA |
| enables double-stranded methylated DNA binding |
IDA
IDA: Inferred from direct assay
|
25258363 | GOA |
| enables hemi-methylated DNA-binding |
IDA
IDA: Inferred from direct assay
|
25258363 | GOA |
| enables protein binding |
IPI
IPI: Inferred from physical interaction
|
7720589 | GOA |
| enables sequence-specific DNA binding |
IDA
IDA: Inferred from direct assay
|
1662794 | GOA |
| enables transcription cis-regulatory region binding |
IDA
IDA: Inferred from direct assay
|
1332065 | GOA |
| enables zinc ion binding |
IDA
IDA: Inferred from direct assay
|
15518539 | GOA |
| Cellular Component GO Annotation | Evidence | References | Source |
|---|---|---|---|
| located in nuclear speck |
IDA
IDA: Inferred from direct assay
|
9553041 | GOA |
| located in nucleoplasm |
IDA
IDA: Inferred from direct assay
|
15520190 | GOA |
| located in nucleus |
IDA
IDA: Inferred from direct assay
|
1662794 | GOA |
WT1 Protein Structure
WT1: Wilm's tumour protein (69 - 389)
zf-H2C2_2: Zinc-finger double domain (407 - 433)
zf-H2C2_2: Zinc-finger double domain (438 - 461)
zf-C2H2: Zinc finger, C2H2 type (482 - 506)
- 0
- 100
- 200
- 300
- 400
- 517 a.a.
| Protein Preferred Names | Protein Names | |
|---|---|---|
|
Wilms tumor protein |
|
WT1 Protein-protein interaction Information
|
Type
|
Protein Name | Protein ID | Interactor | Interactor Species | Interactor ID | Detection Method | References |
|---|---|---|---|---|---|---|---|
|
Intra
|
WT1 | P19544 | KRT40 | Homo sapiens | Q6A162 | 31515488 | |
|
Intra
|
WT1 | P19544 | KRT40 | Homo sapiens | Q6A162 | 25416956 | |
|
Intra
|
WT1 | P19544 | KRTAP10-8 | Homo sapiens | P60410 | 25416956 | |
|
Intra
|
WT1 | P19544 | KRTAP10-8 | Homo sapiens | P60410 | 25416956 | |
|
Intra
|
WT1 | P19544 | TET2 | Homo sapiens | Q6N021 | 25601757 | |
|
Intra
|
WT1 | P19544 | TET2 | Homo sapiens | Q6N021 | 25601757 | |
|
Intra
|
WT1 | P19544 | DVL3 | Homo sapiens | Q92997 | 19447967 |
Recombinant WT1 Proteins
| Cat. No. | Product Name | Accession | Purity |
|---|---|---|---|
| HY-P72261 | Wilms tumor protein/WT1 Protein, Human (His-SUMO) | P19544 (M1-L449) | ≥ 90%, as determined by reducing SDS-PAGE. |
WT1 Antibodies
| Cat. No. | Product Name | Application | Reactivity |
|---|---|---|---|
| HY-P82532 | Wilms Tumor Protein Antibody (YA2277) | WB, IHC-P, ICC/IF, FC | Human, Mouse |
| HY-P82532A | Wilms Tumor Protein Antibody (YA2277)(PBS only) | WB, IHC-P, ICC/IF, FC | Human, Mouse |
| HY-P84159 | Wilms Tumor Protein Antibody (YA3856) | WB, IHC-P, ICC/IF, FC, ELISA | Human, Monkey |
| HY-P84159A | Wilms Tumor Protein Antibody (YA3856)(PBS only) | WB, IHC-P, ICC/IF, FC, ELISA | Human, Monkey |
| HY-P85931 | Wilms Tumor Protein Antibody (YA5623) | IHC-P, WB, ICC/IF, ELISA | Human, Mouse, Rat, Pig |
| HY-P86440 | Wilms Tumor Protein Antibody (YA6132) | WB, IHC-P, ICC/IF, IP, ELISA | Human, Mouse, Rat |
| HY-P86923 | Wilms Tumor Protein Antibody(YA6616) | mIHC | Mouse |
Related Diseases
| Diseases | Alias | |
|---|---|---|
| Wilms Tumor 1 |
|
|
| Denys-Drash Syndrome |
|
|
| Frasier Syndrome |
|
|
| Meacham Syndrome |
|
|
| Nephrotic Syndrome, Type 4 |
|
|
| Mesothelioma, Malignant |
|
|
| Wilms Tumor, Aniridia, Genitourinary Anomalies, And Mental Retardation Syndrome |
|
|
| Diffuse Mesangial Sclerosis |
|
|
| Meacham Winn Culler Syndrome |
|
|
| Aniridia 1 |
|
|
| Desmoplastic Small Round Cell Tumor |
|
|
| Focal Segmental Glomerulosclerosis |
|
|
| Genetic Steroid-Resistant Nephrotic Syndrome |
|
|
| Disorder Of Sexual Development |
|
|
| Gonadoblastoma |
|
|
| Adenomatoid Tumor |
|
|
| Acute Leukemia |
|
|
| Congenital Mesoblastic Nephroma |
|
|
| Clear Cell Sarcoma |
|
|
| 46,Xy Sex Reversal |
|
|
| Premature Menopause |
|
|
| Nephrotic Syndrome |
|
|
| Benign Mesothelioma |
|
|
| Sex Cord-Gonadal Stromal Tumor |
|
|
| Pseudohermaphroditism |
|
|
| Septate Vagina |
|
|
| Hypospadias |
|
|
| Nephroma |
|
|
| 46,Xy Partial Gonadal Dysgenesis |
|
|
| Leukemia |
|
|
| Ewing Sarcoma |
|
|
| Myelodysplastic Syndrome |
|
|
| Myeloid Leukemia |
|
|
| Rhabdoid Cancer |
|
|
| Hereditary Wilms' Tumor |
|
|
| Peritoneum Cancer |
|
|
| Gonadal Dysgenesis |
|
|
| Testicular Gonadoblastoma |
|
|
| Sarcoma |
|
|
| Sertoli-Leydig Cell Tumor |
|
|
| Sarcomatoid Mesothelioma |
|
|
| Sertoli Cell Tumor |
|
|
| Leiomyosarcoma |
|
|
| Chronic Leukemia |
|
|
| Wilms Tumor 5 |
|
|
| Kidney Disease |
|
|
| Dopamine Beta-Hydroxylase Deficiency |
|
|
| Nephrotic Syndrome, Type 1 |
|
|
| Ovarian Brenner Tumor |
|
|
| Epithelial Predominant Wilms' Tumor |
|
|
| Nephrogenic Adenofibroma |
|
|
| Intellectual Developmental Disorder, X-Linked, Syndromic, Wilson-Turner Type |
|
|
| Iris Disease |
|
|
| Metanephric Adenoma |
|
|
| Endosalpingiosis |
|
|
| Renal Adenoma |
|
|
| Thymic Carcinoma |
|
|
| Peritoneal Mesothelioma |
|
|
| Leukemia, Acute Myeloid |
|
|
| Rhabdomyosarcoma 2 |
|
|
| Hobnail Hemangioma |
|
|
| Asbestosis |
|
|
| Cryptorchidism, Unilateral Or Bilateral |
|
|
| Carcinosarcoma |
|
|
| Hematologic Cancer |
|
|
| Fallopian Tube Carcinoma |
|
|
| Malignant Biphasic Mesothelioma |
|
|
| Sarcomatosis |
|
|
| Peritoneal Serous Adenocarcinoma |
|
|
| Neurofibroma Of The Heart |
|
|
| Pericardium Cancer |
|
|
| Pericardial Mesothelioma |
|
|
| Kidney Cortex Disease |
|
|
| Fallopian Tube Squamous Cell Carcinoma |
|
|
| Ovarian Sex-Cord Stromal Tumor |
|
|
| Dysgerminoma |
|
|
| Familial Nephrotic Syndrome |
|
|
| Diaphragmatic Hernia, Congenital |
|
|
| Leukemia, Chronic Myeloid |
|
|
| Cystadenofibroma |
|
|
| Peritoneal Serous Papillary Adenocarcinoma |
|
|
| Ovarian Seromucinous Carcinoma |
|
|
| Spermatic Cord Cancer |
|
|
| Kidney Rhabdoid Cancer |
|
|
| Serous Cystadenocarcinoma |
|
|
| Embryonal Rhabdomyosarcoma |
|
|
| Kidney Cancer |
|
|
| Beckwith-Wiedemann Syndrome |
|
|
| Angiokeratoma Of Fordyce |
|
|
| Ovarian Mesodermal Adenosarcoma |
|
|
| Fallopian Tube Adenocarcinoma |
|
|
| Ovarian Benign Neoplasm |
|
|
| Syndromic X-Linked Intellectual Disability |
|
|
| Kidney Sarcoma |
|
|
| Juvenile Type Testicular Granulosa Cell Tumor |
|
|
| Ovarian Wilms' Cancer |
|
|
| Kidney Benign Neoplasm |
|
|
| Cervical Serous Adenocarcinoma |
|
|
| Hypereosinophilic Syndrome, Idiopathic |
|
|
| Testicular Granulosa Cell Tumor |
|
|
| Ovarian Large-Cell Neuroendocrine Carcinoma |
|
|
| Cauda Equina Neoplasm |
|
|
| Botryoid Rhabdomyosarcoma |
|
|
| Ovarian Cancer |
|
|
| Angioma Serpiginosum |
|
|
| Pierson Syndrome |
|
|
| Hemihyperplasia, Isolated |
|
|
| 46,Xy Sex Reversal 2 |
|
|
| Papillary Serous Adenocarcinoma |
|
|
| Renal Wilms' Tumor |
|
|
| Oral Tuberculosis |
|
|
| Childhood Acute Myeloid Leukemia |
|
|
| Adenofibroma |
|
|
| Ovarian Small Cell Carcinoma |
|
|
| Leukemia, Acute Lymphoblastic |
|
|
| Epididymis Adenocarcinoma |
|
|
| 46,Xx Sex Reversal |
|
|
| Epididymis Cancer |
|
|
| Endometrial Cancer |
|
|
| Fallopian Tube Serous Adenocarcinoma |
|
|
| Small Intestinal Sarcoma |
|
|
| Myelofibrosis |
|
|
| Ovarian Mucinous Neoplasm |
|
|
| Heart Cancer |
|
|
| Endometrioid Ovary Carcinoma |
|
|
| Hematocele Of Tunica Vaginalis Testis |
|
|
| Fallopian Tube Carcinosarcoma |
|
|
| Persistent Mullerian Duct Syndrome |
|
|
| Ovary Serous Adenocarcinoma |
|
|
| Microinvasive Cervical Squamous Cell Carcinoma |
|
|
| Epididymal Neoplasm |
|
|
| Synovium Cancer |
|
|
| Ovarian Mixed Germ Cell Neoplasm |
|
|
| Galloway-Mowat Syndrome 1 |
|
|
| Spindle Cell Hemangioma |
|
|
| Chronic Kidney Disease |
|
|
| Renal Cell Carcinoma, Nonpapillary |
|
|
| Bladder Clear Cell Adenocarcinoma |
|
|
| Hepatoblastoma |
|
|
| Endometrial Stromal Tumor |
|
|
| Lipoid Nephrosis |
|
|
| Myxoid Chondrosarcoma |
|
|
| Renal Hypoplasia |
|
|
| Mixed Cell Type Cancer |
|
|
| Syndromic Intellectual Disability |
|
|
| Endometrial Serous Adenocarcinoma |
|
|
| Angiokeratoma |
|
|
| Thymus Sarcomatoid Carcinoma |
|
|
| Ovarian Serous Carcinoma |
|
|
| Hydrocele |
|
|
| Ovarian Carcinosarcoma |
|
|
| Skin Hemangioma |
|
|
| Childhood Leukemia |
|
|
| Prostate Leiomyoma |
|
|
| Diaphragm Disease |
|
|
| Papillary Cystadenocarcinoma |
|
|
| Cystadenocarcinoma |
|
|
| Ovarian Germ Cell Cancer |
|
|
| Pleural Cancer |
|
|
| Gillespie Syndrome |
|
|
| Kidney Clear Cell Sarcoma |
|
|
| Malignant Teratoma |
|
|
| Female Reproductive Endometrioid Cancer |
|
|
| Testicular Fibroma |
|
|
| Cervical Adenosarcoma |
|
|
| Renal Hypodysplasia/Aplasia 1 |
|
|
| Ovarian Clear Cell Carcinoma |
|
|
| Muscle Cancer |
|
|
| Skeletal Muscle Cancer |
|
|
| Angiokeratoma Circumscriptum |
|
|
| Sarcoma, Synovial |
|
|
| Endometrial Stromal Sarcoma |
|
|
| Ovary Adenocarcinoma |
|
|
| Malignant Ovarian Surface Epithelial-Stromal Neoplasm |
|
|
| 46,Xy Sex Reversal 9 |
|
|
| Rhabdomyosarcoma |
|
|
| Chondrosarcoma, Extraskeletal Myxoid |
|
|
| Liver Leiomyosarcoma |
|
|
| Acute Myeloid Leukemia With Bcr-Abl1 |
|
|
| Inguinal Hernia |
|
|
| Galloway-Mowat Syndrome |
|
|
| Uveal Disease |
|
|
| Alport Syndrome |
|
|
| Chromosomal Deletion Syndrome |
|
|
| Ovarian Gonadoblastoma |
|
|
| Cakut |
|
|
| Ovarian Serous Cystadenofibroma |
|
|
| Parkinson Disease 17 |
|
|
| Benign Peritoneal Mesothelioma |
|
|
| Epithelioid Cell Synovial Sarcoma |
|
|
| Renal Cell Carcinoma, Papillary, 1 |
|
|
| Spermatogenic Failure |
|
|
| Breast Cancer |
|
|
| Papillary Adenofibroma |
|
|
| Acute Promyelocytic Leukemia |
|
|
| Retinitis Pigmentosa |
|
|
Orthologs Information
| Species | Symbol | Source | ID |
|---|---|---|---|
| Felis catus | WT1 | VGNC | VGNC:67095 |
| Mus musculus | WT1 | MGD | MGI:98968 |
| Canis familiaris | WT1 | VGNC | VGNC:48438 |
| Bos taurus | WT1 | VGNC | VGNC:59373 |
| Rattus norvegicus | WT1 | RGD | RGD:3974 |
| Macaca mulatta | WT1 | VGNC | VGNC:78804 |
| Others | WT1 | NCBI |