Wilms’ tumor protein 1 (WT1)

WT1 encodes a zinc finger transcription factor with an N‑terminal proline/glutamine-rich transactivation domain and a C‑terminal region of four C2H2 zinc fingers that bind DNA; alternative splicing and a non-AUG (CUG) upstream start codon generate multiple isoforms with distinct functions[1][3]. WT1 is essential for urogenital development and podocyte maintenance; germline or somatic variants cause pediatric kidney cancers (Wilms tumor) and syndromes such as WAGR, Denys-Drash, and Frasier[5]. In cancer, WT1 exhibits context-dependent behavior, acting as a tumor suppressor when mutated (e.g., Wilms tumor) or functioning as an oncogenic driver when aberrantly overexpressed (e.g., subsets of leukemias and sarcomas), and it is widely used as a diagnostic and monitoring biomarker[2][3][1]. WT1 protein expression is commonly detected by immunohistochemistry in mesothelioma and ovarian serous carcinoma, aiding differential diagnosis, with the caveat that diagnostic interpretation should rely on nuclear staining[1][6]. Experimental data also link WT1 to regulation of growth factor pathways (IGF, TGF-β) and to interaction with other regulators such as p53[4][1].

Other names

Wilms tumor proteinWT33WT1 transcription factorWT1_HUMANWIT-2

Immunotherapeutic targeting of WT1-expressing tumor cells (e.g., peptide vaccine–induced T cell responses or engineered T cells recognizing WT1; inference based on its use as leukemia-associated antigen in the literature; not detailed in the provided sources)