WT1
MCID: HRD009
MIFTS: 31

Hereditary Wilms' Tumor (WT1)

Categories: Blood diseases, Cancer diseases, Fetal diseases, Genetic diseases, Nephrological diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Hereditary Wilms' Tumor

MalaCards integrated aliases for Hereditary Wilms' Tumor:

Name: Hereditary Wilms' Tumor 12 15 70
Hereditary Wilms' Tumour 12
Hereditary Wilms Tumour 12
Hereditary Wilms Tumor 12
Wt1 12

Classifications:



External Ids:

Disease Ontology 12 DOID:5183
NCIt 50 C8496
UMLS 70 C0677779

Summaries for Hereditary Wilms' Tumor

Disease Ontology : 12 A nephroblastoma that results in either bilateral disease or a family history of Wilms' tumour.

MalaCards based summary : Hereditary Wilms' Tumor, also known as hereditary wilms' tumour, is related to wilms tumor 1 and wilms tumor, aniridia, genitourinary anomalies, and mental retardation syndrome, and has symptoms including abdominal pain An important gene associated with Hereditary Wilms' Tumor is PAX6 (Paired Box 6), and among its related pathways/superpathways are Mesodermal Commitment Pathway and Protein ubiquitination. Affiliated tissues include kidney, myeloid and ovary, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Related Diseases for Hereditary Wilms' Tumor

Diseases in the Hereditary Wilms' Tumor family:

Wilms Tumor 1 Wilms Tumor 2
Wilms Tumor 3 Wilms Tumor 4
Wilms Tumor 5 Wilms Tumor 6
Familial Wilms Tumor 2

Diseases related to Hereditary Wilms' Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 506)
# Related Disease Score Top Affiliating Genes
1 wilms tumor 1 31.8 WT1 PAX6 CDC73
2 wilms tumor, aniridia, genitourinary anomalies, and mental retardation syndrome 31.3 WT1 PAX6 LOC106014249 ELP4
3 pax6-related aniridia 31.3 WT1 PAX6
4 aniridia 1 31.2 WT1 PAX6 LOC106014249 ELP4
5 dopamine beta-hydroxylase deficiency 31.2 WT1 PAX6
6 uveal disease 31.0 WT1 PAX6
7 iris disease 31.0 WT1 PAX6 ELP4
8 wilms tumor predisposition 30.9 WT1 CDC73
9 anterior segment dysgenesis 5 29.6 PAX6 ELP4
10 isolated aniridia 29.4 WT1 PAX6 ELP4
11 frasier syndrome 11.6
12 desmoplastic small round cell tumor 11.5
13 denys-drash syndrome 11.5
14 nephrotic syndrome, type 4 11.3
15 leukemia 11.2
16 myeloid leukemia 11.2
17 adenomatoid tumor 11.2
18 leukemia, acute myeloid 11.2
19 focal segmental glomerulosclerosis 11.2
20 acute leukemia 11.2
21 nephrotic syndrome 11.2
22 kidney disease 11.1
23 myelodysplastic syndrome 11.1
24 46,xy sex reversal 11.1
25 breast cancer 11.1
26 diffuse mesangial sclerosis 11.1
27 familial wilms tumor 2 11.1
28 pseudohermaphroditism 11.1
29 myelofibrosis 11.1
30 endometrial cancer 11.1
31 ovarian cancer 11.1
32 sarcoma 11.1
33 mesothelioma, malignant 11.1
34 gonadoblastoma 11.0
35 premature menopause 11.0
36 ewing sarcoma 11.0
37 hypospadias 11.0
38 genetic steroid-resistant nephrotic syndrome 11.0
39 leukemia, acute lymphoblastic 11.0
40 leukemia, chronic myeloid 11.0
41 meacham syndrome 11.0
42 hepatocellular carcinoma 11.0
43 clear cell sarcoma 11.0
44 chronic leukemia 11.0
45 leiomyosarcoma 11.0
46 malignant pleural mesothelioma 11.0
47 chronic kidney disease 11.0
48 gonadal dysgenesis 11.0
49 gastric cancer 11.0
50 benign mesothelioma 11.0

Graphical network of the top 20 diseases related to Hereditary Wilms' Tumor:



Diseases related to Hereditary Wilms' Tumor

Symptoms & Phenotypes for Hereditary Wilms' Tumor

UMLS symptoms related to Hereditary Wilms' Tumor:


abdominal pain

GenomeRNAi Phenotypes related to Hereditary Wilms' Tumor according to GeneCards Suite gene sharing:

26 (show all 23)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.98 WT1
2 Increased shRNA abundance (Z-score > 2) GR00366-A-104 9.98 CTR9 ELP4
3 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.98 WT1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-123 9.98 ELP4
5 Increased shRNA abundance (Z-score > 2) GR00366-A-137 9.98 LEO1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-139 9.98 ELP4
7 Increased shRNA abundance (Z-score > 2) GR00366-A-145 9.98 WT1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.98 WT1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-163 9.98 ELP4
10 Increased shRNA abundance (Z-score > 2) GR00366-A-195 9.98 CTR9
11 Increased shRNA abundance (Z-score > 2) GR00366-A-208 9.98 ELP4
12 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.98 CTR9 LEO1 WT1
13 Increased shRNA abundance (Z-score > 2) GR00366-A-216 9.98 ELP4
14 Increased shRNA abundance (Z-score > 2) GR00366-A-24 9.98 CTR9
15 Increased shRNA abundance (Z-score > 2) GR00366-A-30 9.98 WT1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-40 9.98 LEO1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.98 WT1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.98 WT1
19 Increased shRNA abundance (Z-score > 2) GR00366-A-55 9.98 LEO1
20 Increased shRNA abundance (Z-score > 2) GR00366-A-6 9.98 ELP4
21 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.98 LEO1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.98 WT1
23 Increased shRNA abundance (Z-score > 2) GR00366-A-91 9.98 CTR9

Drugs & Therapeutics for Hereditary Wilms' Tumor

Search Clinical Trials , NIH Clinical Center for Hereditary Wilms' Tumor

Genetic Tests for Hereditary Wilms' Tumor

Anatomical Context for Hereditary Wilms' Tumor

MalaCards organs/tissues related to Hereditary Wilms' Tumor:

40
Kidney, Myeloid, Ovary, Lung, Breast, Bone, Liver

Publications for Hereditary Wilms' Tumor

Articles related to Hereditary Wilms' Tumor:

# Title Authors PMID Year
1
Hereditary hyperparathyroidism-jaw tumor syndrome: the endocrine tumor gene HRPT2 maps to chromosome 1q21-q31. 61
7717405 1995
2
WT1 mutations contribute to abnormal genital system development and hereditary Wilms' tumour. 61
1654525 1991
3
An estimate of the heritable fraction of childhood cancer. 61
2069856 1991
4
Tumor-specific loss of 11p15.5 alleles in del11p13 Wilms tumor and in familial adrenocortical carcinoma. 61
2566168 1989
5
Heritable fraction of unilateral Wilms tumor. 61
2827098 1988

Variations for Hereditary Wilms' Tumor

Expression for Hereditary Wilms' Tumor

Search GEO for disease gene expression data for Hereditary Wilms' Tumor.

Pathways for Hereditary Wilms' Tumor

Pathways related to Hereditary Wilms' Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.58 RTF1 PAX6 LEO1 ELP4 CTR9 CDC73
2
Show member pathways
11.52 RTF1 LEO1 CTR9 CDC73

GO Terms for Hereditary Wilms' Tumor

Cellular components related to Hereditary Wilms' Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 9.76 WT1 RTF1 PAX6 NYNRIN LEO1 ELP4
2 nucleoplasm GO:0005654 9.5 WT1 RTF1 PAX6 LEO1 ELP4 CTR9
3 Cdc73/Paf1 complex GO:0016593 8.92 RTF1 LEO1 CTR9 CDC73

Biological processes related to Hereditary Wilms' Tumor according to GeneCards Suite gene sharing:

(show all 20)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 9.91 WT1 RTF1 PAX6 CTR9 CDC73
2 protein ubiquitination GO:0016567 9.86 RTF1 LEO1 CTR9 CDC73
3 Wnt signaling pathway GO:0016055 9.76 RTF1 LEO1 CTR9 CDC73
4 transcription by RNA polymerase II GO:0006366 9.73 RTF1 LEO1 CTR9 CDC73
5 negative regulation of epithelial cell proliferation GO:0050680 9.59 PAX6 CDC73
6 camera-type eye development GO:0043010 9.58 WT1 PAX6
7 mRNA polyadenylation GO:0006378 9.58 LEO1 CDC73
8 negative regulation of myeloid cell differentiation GO:0045638 9.58 LEO1 CTR9 CDC73
9 beta-catenin-TCF complex assembly GO:1904837 9.57 LEO1 CDC73
10 positive regulation of histone H3-K4 methylation GO:0051571 9.55 RTF1 CTR9
11 histone H3-K4 trimethylation GO:0080182 9.54 RTF1 CTR9
12 histone monoubiquitination GO:0010390 9.54 LEO1 CTR9 CDC73
13 positive regulation of transcription elongation from RNA polymerase II promoter GO:0032968 9.52 LEO1 CDC73
14 histone modification GO:0016570 9.5 LEO1 CTR9 CDC73
15 positive regulation of mRNA 3'-end processing GO:0031442 9.49 LEO1 CDC73
16 blastocyst growth GO:0001832 9.48 RTF1 CTR9
17 stem cell population maintenance GO:0019827 9.46 RTF1 LEO1 CTR9 CDC73
18 histone H2B ubiquitination GO:0033523 9.43 LEO1 CTR9 CDC73
19 transcription elongation from RNA polymerase II promoter GO:0006368 9.35 RTF1 LEO1 ELP4 CTR9 CDC73
20 endodermal cell fate commitment GO:0001711 8.92 RTF1 LEO1 CTR9 CDC73

Molecular functions related to Hereditary Wilms' Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 RNA polymerase II C-terminal domain phosphoserine binding GO:1990269 8.96 RTF1 LEO1
2 RNA polymerase II complex binding GO:0000993 8.8 ELP4 CTR9 CDC73

Sources for Hereditary Wilms' Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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