Anti-WT1 Rabbit Monoclonal (Clone RM516)

Wilms tumor protein 1 (WT1) is a transcription factor and member of the SP1 family of transcription factors. It is composed of an N-terminal dimerization domain, a transcriptional repressor domain, a transactivation domain, and a C-terminal DNA/RNA-binding domain containing four zinc-finger motifs.2,1 There are many isoforms of WT1 formed by various processes. The most common isoforms contain or lack a lysine, threonine, and serine sequence (+KTS or -KTS) in the zinc-finger region, contain or lack 17 amino acids encoded by exon 5 (+17aa or -17aa), lack the KTS sequence but contain the 17-amino acid sequence (-KTS/+17aa), or contain both sequences (+KTS/+17aa).3,1 WT1 is expressed in, and crucial for the development of, the genitourinary system, gonads, and adrenal glands. WT1 binds to DNA and acts as a transcriptional repressor or activator in a context-dependent manner to modulate the expression of its targets, including NR5A1/SF1, which is involved in adrenal gland and gonadal development. It also binds to and modulates mRNA splicing and stability.1 Certain mutations in WT1 disrupt differentiation of metanephric mesenchyme cells and are associated with Wilms tumor, a renal cancer primarily affecting children.4 Other WT1 mutations are associated with non-cancer pathologies, such as Frasier syndrome and Denys-Drash syndrome, which include genitourinary and gonadal development disruptions, as well as heart and central and peripheral nervous system disorders. Cayman's WT1 Rabbit Monoclonal Antibody (Clone RM516) can be used for immunohistochemistry (IHC) and Western blot (WB) applications. Protein function: Transcription factor that plays an important role in cellular development and cell survival (PubMed:7862533). Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3' (PubMed:7862533, PubMed:17716689, PubMed:25258363). 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 (PubMed:15520190). Isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing (PubMed:16934801). Isoform 1 has lower affinity for DNA, and can bind RNA (PubMed:19123921). [The UniProt Consortium]