Tumour predisposition - childhood onset

Gene: WT1

Green List (high evidence)

WT1 (Wilms tumor 1)
EnsemblGeneIds (GRCh38): ENSG00000184937
EnsemblGeneIds (GRCh37): ENSG00000184937
OMIM: 607102, Gene2Phenotype
WT1 is in 21 panels

3 reviews

Ivone Leong (Genomics England Curator)

Green List (high evidence)

As discussed at the Genomics Cancer Panel Workshop, 16th July 2019: the group agreed that there is enough evidence to rate this gene green
Created: 2 Aug 2019, 11:03 a.m. | Last Modified: 2 Aug 2019, 11:03 a.m.
Panel Version: 1.27

Lara Hawkes (Genomics England)

Green List (high evidence)

Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

Phenotypes
Familial Wilms tumor

Richard Scott (North Thames GMC/UCL)

Green List (high evidence)

Some (most) missense mutations in zinc-finger domain are disease causing but rarely inherited, so de novo status is the best handle. PMID 15483024 is a good source.
Created: 6 Aug 2015, 3:55 a.m.

Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

Publications

Mode of pathogenicity
loss-of-function (truncating variants and curated list of variants)

Details

Mode of Inheritance
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Sources
  • NHS GMS
  • Expert List
  • Expert Review Green
  • Eligibility statement prior genetic testing
  • UKGTN
  • Emory Genetics Laboratory
  • Illumina TruGenome Clinical Sequencing Services
  • Radboud University Medical Center, Nijmegen
Phenotypes
  • Wagner Syndrome
  • Frasier syndrome, 136680
  • Nephrotic syndrome, type 4, 256370
  • Denys-Drash syndrome, 194080
  • Familial Wilms tumor
  • Wilms Tumor 1
  • Wilms Tumor
  • Wilms tumour
  • Meacham syndrome, 608978
  • Mesothelioma, somatic, 156240
  • Denys-Drash Syndrome
  • Wilms tumor, type 1, 194070
OMIM
607102
Clinvar variants
Variants in WT1
Penetrance
Complete
Publications
Panels with this gene

History Filter Activity

2 Aug 2019, Gel status: 3

Added New Source, Set mode of inheritance, Set Phenotypes, Set publications

Ivone Leong (Genomics England Curator)

Source NHS GMS was added to WT1. Mode of inheritance for gene WT1 was changed from MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown Added phenotypes Familial Wilms tumor for gene: WT1 Publications for gene WT1 were changed from 15483024 to 23788249

30 Jan 2019, Gel status: 3

Added New Source, Status Update

Ivone Leong (Genomics England Curator)

Source Expert List was added to WT1. Rating Changed from Green List (high evidence) to Green List (high evidence)

14 Feb 2016, Gel status: 4

Gene classified by Genomics England curator

Ellen Thomas (Genomics England Curator)

This gene has been classified as Green List (High Evidence).

14 Feb 2016, Gel status: 4

Set publications

Ellen Thomas (Genomics England Curator)

Publications for WT1 were set to 15483024

14 Feb 2016, Gel status: 4

Set Mode of Inheritance

Ellen Thomas (Genomics England Curator)

Mode of inheritance for WT1 was changed to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

28 Aug 2015, Gel status: 4

Upload gene information

Ellen McDonagh (Genomics England Curator)

WT1 was added to Paediatric congenital malformation-dysmorphism-tumour syndromes panel. Sources: Eligibility statement prior genetic testing,Radboud University Medical Center, Nijmegen,Illumina TruGenome Clinical Sequencing Services,Emory Genetics Laboratory,UKGTN

28 Aug 2015, Gel status: 4

Set Phenotypes

Ellen McDonagh (Genomics England Curator)

Phenotypes for gene WT1 were set to Wilms tumor, type 1, 194070; Denys-Drash syndrome, 194080; Nephrotic syndrome, type 4, 256370; Frasier syndrome, 136680; Meacham syndrome, 608978; Mesothelioma, somatic, 156240;Wagner Syndrome;Wilms Tumor;Wilms Tumor 1;Denys-Drash Syndrome; Wilms tumour

22 Jul 2015, Gel status: 4

Set Mode of Inheritance

Eik Haraldsdottir (Genomics England)

Model of inheritance for gene WT1 was changed to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

22 Jul 2015, Gel status: 4

Set Mode of Inheritance

Eik Haraldsdottir (Genomics England)

Model of inheritance for gene WT1 was changed to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

22 Jul 2015, Gel status: 4

Set Mode of Inheritance

Eik Haraldsdottir (Genomics England)

Model of inheritance for gene WT1 was changed to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

22 Jul 2015, Gel status: 4

Set Mode of Inheritance

Eik Haraldsdottir (Genomics England)

Model of inheritance for gene WT1 was changed to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

22 Jul 2015, Gel status: 4

Added New Source

Eik Haraldsdottir (Genomics England)

WT1 was added to Paediatric congenital malformation-dysmorphism-tumour syndromes panel. Sources: UKGTN

22 Jul 2015, Gel status: 3

Added New Source

Eik Haraldsdottir (Genomics England)

WT1 was added to Paediatric congenital malformation-dysmorphism-tumour syndromes panel. Sources: Emory Genetics Laboratory

22 Jul 2015, Gel status: 2

Set Mode of Inheritance

Eik Haraldsdottir (Genomics England)

Model of inheritance for gene WT1 was changed to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

22 Jul 2015, Gel status: 2

Set Mode of Inheritance

Eik Haraldsdottir (Genomics England)

Model of inheritance for gene WT1 was changed to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

22 Jul 2015, Gel status: 2

Added New Source

Eik Haraldsdottir (Genomics England)

WT1 was added to Paediatric congenital malformation-dysmorphism-tumour syndromes panel. Sources: Illumina TruGenome Clinical Sequencing Services

22 Jul 2015, Gel status: 1

Added New Source

Eik Haraldsdottir (Genomics England)

WT1 was added to Paediatric congenital malformation-dysmorphism-tumour syndromes panel. Sources: Radboud University Medical Center, Nijmegen