Immunogen
Synthesized peptide derived from human Wilms' Tumor 1(WT1)
Specificity
This antibody detects endogenous levels of human Wilms' Tumor 1(WT1). Heat-induced epitope retrieval (HIER) TRIS-EDTA of pH8.0 was highly recommended as antigen repair method in paraffin section
Source
Mouse, Monoclonal/IgG2b, Kappa
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 500-2000 IHC-p 1:100-500. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
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. [provided by RefSeq, Mar 2015],
Function
disease:A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;22)(p13;q12) with EWSR1.,disease:Defects in WT1 are a cause of hypospadias. Hypospadias is a common malformation in which the urethra opens on the ventral side of the penis. It is considered a complex disorder with both genetic and environmental factors involved in the pathogenesis. Hypospadias can occur alone on an apparently multifactorial basis or as part of syndromes.,disease:Defects in WT1 are a cause of Meacham syndrome [MIM:608978]. Meacham syndrome is a rare sporadically occurring multiple malformation syndrome characterized by male pseudohermaphroditism with abnormal internal female genitalia comprising a uterus and double or septate vagina, complex congenital heart defect and diaphragmatic abnormalities.,disease:Defects in WT1 are a cause of Wilms tum
Protein name
Wilms tumor protein (WT33)
Abbreviation
Wilms' Tumor 1
Fields
>>Transcriptional misregulation in cancer
Human protein sequence Database
P19544
Mouse protein sequence database
Rat protein sequence database
Cellular localization
Nucleus . Nucleus, nucleolus. Cytoplasm . Isoforms lacking the KTS motif have a diffuse nuclear location (PubMed:15520190). Shuttles between nucleus and cytoplasm. .; [Isoform 1]: Nucleus speckle .; [Isoform 4]: Nucleus, nucleoplasm .
Tissue expression
Expressed in the kidney and a subset of hematopoietic cells.
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