Overview

Location [1]
11p13
Pathway
Beta-Catenin/WNT signaling
Protein [2]
Wilms tumor protein
Synonyms [1]
GUD, WAGR, WIT-2, WT33, NPHS4, AWT1

Wilms tumor 1 (WT1) is a gene that encodes a transcription factor that contains four zinc finger motifs and a proline/glutamine-rich DNA binding region at opposite termini. Fusions, missense mutations, nonsense mutations, silent mutations and frameshift deletions are observed in cancers such as acute lymphoblastic leukemia, chronic myeloid leukemia, kidney cancer, lung cancer, and skin cancer.

WT1 is altered in 1.45% of all cancers with lung adenocarcinoma, breast invasive ductal carcinoma, acute myeloid leukemia, colon adenocarcinoma, and desmoplastic small round cell tumor having the greatest prevalence of alterations [3].

WT1 GENIE Cases - Top Diseases

The most common alterations in WT1 are WT1 Mutation (1.41%), WT1 Amplification (0.25%), WT1 Loss (0.03%), WT1 P136S (0.02%), and WT1 G60R (0.02%) [3].

WT1 GENIE Cases - Top Alterations

Significance of WT1 in Diseases

Acute Myeloid Leukemia +

Ovarian Carcinoma +

Fallopian Tube Carcinoma +

Multiple Myeloma +

Myelodysplastic Syndromes +

Primary Peritoneal Carcinoma +

Myeloid Neoplasm +

Acute Lymphoblastic Leukemia +

Endometrial Carcinoma +

Non-Small Cell Lung Carcinoma +

Mesothelioma +

Non-Hodgkin Lymphoma +

Acute Biphenotypic Leukemia +

Acute Erythroid Leukemia +

Acute Megakaryoblastic Leukemia +

Acute Undifferentiated Leukemia +

Chronic Myeloid Leukemia +

Hodgkin Lymphoma +

Juvenile Myelomonocytic Leukemia +

Malignant Pleural Mesothelioma +

Myelodysplastic Syndrome With Excess Blasts-2 +

Myeloid Sarcoma +

Natural Killer Cell Lymphoblastic Leukemia/Lymphoma +

Secondary Acute Myeloid Leukemia +

References

1. Hart R and Prlic A. Universal Transcript Archive Repository. Version uta_20180821. San Francisco CA: Github;2015. https://github.com/biocommons/uta

2. The UniProt Consortium. UniProt: a worldwide hub of protein knowledge. Nucleic Acids Research. 2019;47:D506-D515.

3. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

4. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.