Recombinant Human Wilms tumor protein (WT1), Truncated

Uniprot Id

P19544

Target Species

Human

Target Name

WT1

Target Full Name

Wilms tumor protein

Target Function

Transcription factor that plays an important role in cellular development and cell survival. Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3'. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may act as transcription factors. Isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing. Isoform 1 has lower affinity for DNA, and can bind RNA.

Target Involvement

Frasier syndrome (FS); Wilms tumor 1 (WT1); Denys-Drash syndrome (DDS); Nephrotic syndrome 4 (NPHS4); Meacham syndrome (MEACHS); Mesothelioma, malignant (MESOM)

Target Subcellular Location

Nucleus. Nucleus, nucleolus. Cytoplasm.; [Isoform 1]: Nucleus speckle.; [Isoform 4]: Nucleus, nucleoplasm.

Target Protein Families

EGR C2H2-type zinc-finger protein family

Target Tissue Specificity

Expressed in the kidney and a subset of hematopoietic cells.

Target Research Area

Cancer, Transcription

Target Synonyms

WIT 2; WT 1; AWT1; FWT1; GUD; NPHS4; WAGR; Wilms tumor 1; Wilms Tumor; Wilms tumor protein; Wilms' tumor gene; Wilms' tumor protein; WIT2; WT; WT1; WT1_HUMAN; WT33

Target Background

This gene encodes a transcription factor that contains four zinc-finger motifs at the C-terminus and a proline/glutamine-rich DNA-binding domain at the N-terminus. It has an essential role in the normal development of the urogenital system, and it is mutated in a small subset of patients with Wilms tumor. This gene exhibits complex tissue-specific and polymorphic imprinting pattern, with biallelic, and monoallelic expression from the maternal and paternal alleles in different tissues. Multiple transcript variants have been described. In several variants, there is evidence for the use of a non-AUG (CUG) translation initiation codon upstream of, and in-frame with the first AUG. Authors of PMID:7926762 also provide evidence that WT1 mRNA undergoes RNA editing in human and rat, and that this process is tissue-restricted and developmentally regulated.