Previous symbols: GUD. Aliases: WAGR, WIT-2, AWT1, NPHS4, WT-1.
ai-gene-review has this gene at status IN_PROGRESS; the description is shown only once the review is complete. Read the draft upstream.
Relationship is the entry's genetic[].relationship_type. untyped means the entry has a genetic record for the gene but does not type it; its free-text association is shown instead.
| Disorder | Relationship | Where the gene appears |
|---|---|---|
| 46,XX testicular disorder of sex development | causative de novo |
mechanism: WT1 C-terminal regulatory alteration
subtype: WT1-related
scoped to subtype: WT1-related
|
| 46,XY partial gonadal dysgenesis | causative germline |
mechanism: WT1 sequence alteration
subtype: WT1-related PGD
|
| Acute Myeloid Leukemia with CEBPA Somatic Mutations | modifier somatic |
scoped to subtype: CEBPA bZIP In-Frame-Indel AML
|
| Aniridia | causative |
mechanism: WT1 Haploinsufficiency in the Contiguous 11p13 Deletion
subtype: WAGR
scoped to subtype: WAGR
|
| Congenital Diaphragmatic Hernia | causative | |
| Denys-Drash Syndrome | causative germline | |
| Frasier Syndrome | causative germline | |
| Mixed Phenotype Acute Leukemia | somatic driver somatic |
mechanism: Immature T-Lineage Program with Retained Myeloid Potential
scoped to subtype: MPAL T/myeloid NOS
|
| Pelvic Organ Prolapse | susceptibility | |
| WT1-Related Wilms Tumor Predisposition |
causative
germlinesomatic
loss of function
CLINGEN: definitive
|
mechanism: Biallelic WT1 Loss in Nephron Progenitors; Constitutional WT1 Haploinsufficiency in Gonadal and Glomerular Development; Germline WT1 Loss-of-Function; Somatic Paternal Uniparental Disomy of 11p
|
| Meacham syndrome |
untyped
Causal heterozygous missense mutation
|
|
| Wilms Tumor |
untyped
Tumor Suppressor Loss
|
mechanism: WT1 Inactivation
|
| WAGR Syndrome | no genetic record |
mechanism: Somatic biallelic WT1 inactivation in renal precursor cells; WT1 haploinsufficiency and Wilms tumor predisposition
|
An entry matches when ClinGen's MONDO disease is the entry's own disease term, a subtype term, or an exact-match mapping. An amber status marks an entry ClinGen supports the gene for (Definitive, Strong or Moderate) whose own record does not type it. A Limited, Disputed or Refuted tier on such an entry is not a gap.
| ClinGen disease | MOI | Classification | dismech entry |
|---|---|---|---|
| Wilms tumor 1 MONDO:0008679 | AD | Definitive 2020-07-30 |
WT1-Related Wilms Tumor Predisposition
causative
|
| Denys-Drash syndrome MONDO:0008682 | AD | Definitive 2021-07-27 |
Denys-Drash Syndrome
causative
|
Identity from HGNC (snapshot 2026-10-01). Page layers are described in the gene pages documentation.