Location [1]
Beta-Catenin/WNT signaling
Protein [2]
Wilms tumor protein
Synonyms [1]

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 1.78% of all cancers with lung adenocarcinoma, breast invasive ductal carcinoma, colon adenocarcinoma, acute myeloid leukemia, 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 (1.25%), WT1 Amplification (0.26%), WT1 Fusion (0.12%), WT1-EWSR1 Fusion (0.11%), and WT1 Loss (0.06%) [3].

WT1 GENIE Cases - Top Alterations

Significance of WT1 in Diseases

Acute Myeloid Leukemia +

Malignant Solid Tumor +

Ovarian Carcinoma +

Myelodysplastic Syndromes +

Non-Hodgkin Lymphoma +

Fallopian Tube Carcinoma +

Multiple Myeloma +

Primary Peritoneal Carcinoma +

Desmoplastic Small Round Cell Tumor +

Acute Lymphoblastic Leukemia +

Soft Tissue Sarcoma +

Chronic Myeloid Leukemia +

Endometrial Carcinoma +

Secondary Acute Myeloid Leukemia +

Myelodysplastic Syndrome With Excess Blasts-2 +

Non-Small Cell Lung Carcinoma +

Myeloid Neoplasm +

Mesothelioma +

Malignant Pleural Mesothelioma +

Acute Biphenotypic Leukemia +

Acute Erythroid Leukemia +

Acute Megakaryoblastic Leukemia +

Acute Undifferentiated Leukemia +

Hodgkin Lymphoma +

Juvenile Myelomonocytic Leukemia +

Myeloid Sarcoma +

Natural Killer Cell Lymphoblastic Leukemia/Lymphoma +


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 8. 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.