Ordering Information
- : MAB4234
- : 100 µg
- : 1 mg/mL
Anti-Wilms' Tumor, N-terminus, nephroblastoma tumor, clone 6F-H2
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | ELISA, IF, WB | Mouse | Purified | Monoclonal Antibody |
Description:
Anti-Wilms' Tumor, N-terminus, nephroblastoma tumor, clone 6F-H2
Trade Name:
Chemicon (Millipore)
Specificity:
The antibody reacts with all isoforms of the full-length WT1 protein. The antibody staining location is nuclear.
Epitope:
N-terminus, nephroblastoma tumor
Immunogen:
aa 1-173 amino acides of the WT-1 protein
Clone:
6F-H2
Isotype:
IgG1κ
Background Information:
Wilm's Tumor (WT), a sporadic and familial childhood kidney tumor, is genetically heterogeneous. Wilms' tumor is associated with mutations of WT1, a zinc-finger transcription factor that is essential for the development of the metanephric kidney and the urogenital system. The WT1 gene is normally expressed in fetal kidney and mesothelium, and its expression has been suggested as a marker for Wilms tumor and mesothelioma.
Species Reactivity:
Human
Application Notes:
Immunofluorescence:
Western Blot:
ELISA:
Optimal working dilutions must be determined by end user.
Western Blot:
ELISA:
Optimal working dilutions must be determined by end user.
Presentation:
Purified immunoglobulin from Protein A Sepharose™ chromatography. Liquid in 0.02M PB, pH 7.6, 0.25M NaCl containing 0.1% sodium azide.
Storage Conditions:
Maintain at 2-8°C in undiluted aliquots for up to 6 months after date of receipt.
UniProt Number:
Entrez Gene Number:
Gene Symbol:
- WT1
- GUD
- WT33
- WAGR
- WIT-2
Alternate Names:
WT1
Usage Statement:
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Key Applications:
- ELISA
- Immunofluorescence
- Western Blotting
Entrez Gene Summary:
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 Wilm's tumors. Multiple transcript variants, resulting from alternative splicing at two coding exons, have been well characterized. There is also evidence for the use of non-AUG (CUG) translation initiation site upstream of, and in-frame with the first AUG, leading to additional isoforms. 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.
UniProt Summary:
FUNCTION: SwissProt: P19544 # Potential role in transcriptional regulation. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'.
SIZE: 449 amino acids; 49188 Da
SUBUNIT: Interacts with ZNF224 via the zinc-finger region. Interacts with WTAP and SRY.
SUBCELLULAR LOCATION: Nucleus.
TISSUE SPECIFICITY: Expressed in the kidney and a subset of hematopoietic cells.
DISEASE: SwissProt: P19544 # Defects in WT1 are the cause of Frasier syndrome (FS) [MIM:136680]. FS is characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys-Drash syndrome. Inheritance is autosomal dominant. & Defects in WT1 are the cause of the genitourinary dysplasia component of WAGR syndrome (GUD) [MIM:137357]. WAGR syndrome is a contiguous gene syndrome characterized by Wilms tumor, aniridia, genitourinary anomalies and mental retardation. These clinical features are due to defects in different genes. & Defects in WT1 are the cause of Wilms tumor 1 (WT1) [MIM:194070]. WT is an embryonal malignancy of the kidney that affects approximately 1 in 10'000 infants and young children. It occurs both in sporadic and hereditary forms. & Defects in WT1 are the cause of Denys-Drash syndrome (DDS) [MIM:194080]. DDS is a typical nephropathy characterized by diffuse mesangial sclerosis, genital abnormalities, and/or Wilms tumor. There is phenotypic overlap with WAGR syndrome and Frasier syndrome. Inheritance is autosomal dominant, but most cases are sporadic. & Defects in WT1 are the cause of isolated diffuse mesangial sclerosis (IDMS) [MIM:256370]. IDMS is an early-onset nephrotic syndrome occurring in the absence of other abnormalities and resulting in renal failure. Inheritance is autosomal recessive. & A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;22)(p13;q12) with EWSR1.
SIMILARITY: SwissProt: P19544 ## Belongs to the EGR C2H2-type zinc-finger protein family. & Contains 4 C2H2-type zinc fingers.
SIZE: 449 amino acids; 49188 Da
SUBUNIT: Interacts with ZNF224 via the zinc-finger region. Interacts with WTAP and SRY.
SUBCELLULAR LOCATION: Nucleus.
TISSUE SPECIFICITY: Expressed in the kidney and a subset of hematopoietic cells.
DISEASE: SwissProt: P19544 # Defects in WT1 are the cause of Frasier syndrome (FS) [MIM:136680]. FS is characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys-Drash syndrome. Inheritance is autosomal dominant. & Defects in WT1 are the cause of the genitourinary dysplasia component of WAGR syndrome (GUD) [MIM:137357]. WAGR syndrome is a contiguous gene syndrome characterized by Wilms tumor, aniridia, genitourinary anomalies and mental retardation. These clinical features are due to defects in different genes. & Defects in WT1 are the cause of Wilms tumor 1 (WT1) [MIM:194070]. WT is an embryonal malignancy of the kidney that affects approximately 1 in 10'000 infants and young children. It occurs both in sporadic and hereditary forms. & Defects in WT1 are the cause of Denys-Drash syndrome (DDS) [MIM:194080]. DDS is a typical nephropathy characterized by diffuse mesangial sclerosis, genital abnormalities, and/or Wilms tumor. There is phenotypic overlap with WAGR syndrome and Frasier syndrome. Inheritance is autosomal dominant, but most cases are sporadic. & Defects in WT1 are the cause of isolated diffuse mesangial sclerosis (IDMS) [MIM:256370]. IDMS is an early-onset nephrotic syndrome occurring in the absence of other abnormalities and resulting in renal failure. Inheritance is autosomal recessive. & A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;22)(p13;q12) with EWSR1.
SIMILARITY: SwissProt: P19544 ## Belongs to the EGR C2H2-type zinc-finger protein family. & Contains 4 C2H2-type zinc fingers.
Brand Family:
Chemicon
Protein/Isoform Description:
Wilm's Tumor (WT), a sporadic and familial childhood kidney tumor, is genetically heterogeneous. Wilm's Tumor is associated with mutations of WT1, a zinc-finger transcription factor that is essential for the development of the metanephric kidney and the urogenital system. The WT1 gene is normally expressed in fetal kidney and mesothelium, and its expression has been suggested as a marker for Wilm's Tumor and mesothelioma.
Product Name:
Anti-Wilms' Tumor, N-terminus, nephroblastoma tumor, clone 6F-H2
Concentration:
1 mg/mL
Antibody Type:
Monoclonal Antibody
Qty/Pk:
100 µg
Format:
Purified
Host:
Mouse