抗体

Wilms瘤抑癌基因

MBS370117

5 + Control Slides

115美元

单克隆

小鼠

未共轭

6F-H2

抗体

Wilms瘤抑癌基因

Wilms瘤抑癌基因

肾母细胞瘤蛋白质亚型D;肾母细胞瘤蛋白;肾母细胞瘤蛋白;OTTHUMP00000037553;OTTHUMP00000037554;OTTHUMP00000037555;OTTHUMP00000037556;OTTHUMP00000238018;肾母细胞瘤1;WT33

Wilms瘤抑癌基因

Wilms瘤抑癌基因;Wilms瘤抑癌基因;GUD;AWT1;WAGR;WT33;NPHS4;机智-2

WT1_HUMAN

单克隆

IgG1/K

6F-H2

小鼠

人类

WT1 is a mouse monoclonal antibody derived from cell culture supernatant that is concentrated, dialyzed, filter sterilized and diluted in buffer pH 7.5, containing BSA and sodium azide as a preservative.

Store at 2 to 8 degree C in the dark.

Control: Malignant Mesothelioma, Kidney, Testicle. Localization: Nuclear

Reactivity Note: Paraffin, Frozen

Wilms' Tumor Protein (WT1) is a suppressor gene located on Chromosome 11p13. Mutations of the WT1 gene on Chromosome 11 are observed in approximately 20% of Wilms tumors. At least half of the Wilms tumors with mutations in WT1 also carry mutations in CTNNB1, the gene encoding the proto-oncogene beta-catenin.

65508004

NP_077744.3

NM_024426.3

Q4VXV5

56150 Da

Regulation Of Telomerase Pathway (137987)

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. 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 site 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]

Function: Transcription factor that plays an important role in cellular development and cell survival. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'. 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. Ref.24 Ref.25. Subunit structure: Homodimer. Interacts with WTIP. Interacts with actively translating polysomes. Detected in nuclear ribonucleoprotein (mRNP) particles. Interacts with HNRNPU via the zinc-finger region. Interacts with U2AF2. Interacts with CITED2. By similarity. Interacts with ZNF224 via the zinc-finger region. Interacts with WTAP and SRY. Interacts with FAM123B/WTX. Ref.16 Ref.17 Ref.18 Ref.25. Subcellular location: Nucleus. Cytoplasm. By similarity. Note: Shuttles between nucleus and cytoplasm. By similarity. Ref.23Isoform 1: Nucleus speckle Ref.23. Isoform 4: Nucleus nucleoplasm Ref.23. Tissue specificity: Expressed in the kidney and a subset of hematopoietic cells. Involvement in disease: 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. Ref.42Defects 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. Ref.28 Ref.38 Ref.39 Ref.47Defects 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. Ref.11 Ref.12 Ref.29 Ref.30 Ref.31 Ref.32 Ref.33 Ref.35 Ref.36 Ref.37 Ref.39 Ref.40 Ref.43 Ref.44 Ref.45 Ref.46 Ref.50Defects in WT1 are the cause of nephrotic syndrome type 4 (NPHS4) [. MIM:256370]. A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. Most patients with NPHS4 show diffuse mesangial sclerosis on renal biopsy, which is a pathologic entity characterized by mesangial matrix expansion with no mesangial hypercellularity, hypertrophy of the podocytes, vacuolized podocytes, thickened basement membranes, and diminished patency of the capillary lumen. Ref.39 Ref.41 Ref.44 Ref.49Defects in WT1 are a cause of Meacham syndrome (MEACHS) [. 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. Ref.51Note=A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;22)(p13;q12) with EWSR1. Miscellaneous: Presence of the KTS motif hinders interactions between DNA and zinc-finger 4. Sequence similarities: Belongs to the EGR C2H2-type zinc-finger protein family.Contains 4 C2H2-type zinc fingers. RNA editing: Edited at position 281.Partially edited. Ref.14. Sequence caution: The sequence AAB33443.1 differs from that shown. Reason: Contaminating sequence. Sequence of unknown origin in the N-terminal part.The sequence CAA35956.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.The sequence CAA35956.1 differs from that shown. Reason: Unusual initiator. The initiator methionine is coded by a non-canonical CTG leucine codon.The sequence CAC39220.3 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.The sequence CAI95758.2 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.The sequence CAI95759.2 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

5 + Control Slides

115美元

3 ml (Prediluted)

160

0.1 mL (Concentrate)

170

7 ml (Prediluted)

195

0.5 mL (Concentrate)

275