Wilms tumor 1
MONDO:0008679Mondo
Findings
No curated finding names Wilms tumor 1 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
An autosomal disorder due to pathogenic variants in the WT1 gene leading to an increased risk Wilms tumor and genitourinary abnormalities incorporating Denys-Drash syndrome and Frasier syndrome.
Definition from the Mondo Disease Ontology (MONDO:0008679), read 2026-09-29. CC BY 4.0.
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- WT1HGNC:12796
- Definitive · ClinGen · Autosomal dominant · 2020
- Definitive · G2P · Autosomal dominant · 2019
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2023
Where it sits
- A kind of
Other names
6 names
Resolves to: Wilms tumor 1
- Also called
- Wilms tumor type 1Wilms tumor, autosomal dominant, somatic mutationWilms tumor, somaticWilms tumor, type 1, autosomal dominant, somatic mutationWilms tumour type 1WT1-related Wilms tumor predisposition