Wilms Tumor Protein,肾母细胞瘤蛋白抗体

产品编号HZ-6983R
英文名称Wilms Tumor Protein
中文名称肾母细胞瘤蛋白抗体
别 名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; WT-1; WT1_HUMAN; WT33.
说 明 书0.1ml 0.2ml
研究领域肿瘤 细胞生物 免疫学 发育生物学 肿瘤细胞生物标志物 表观遗传学
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Sheep,
Wilms Tumor Protein,肾母细胞瘤蛋白抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量55kDa
细胞定位细胞核 细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Wilms Tumor Protein
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Wilms Tumor Protein,肾母细胞瘤蛋白抗体PubMedPubMed
产品介绍background:
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.

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.

Subunit:
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. Interacts with ZNF224 via the zinc-finger region. Interacts with WTAP and SRY. Interacts with FAM123B/WTX. Interacts with RBM4.

Wilms Tumor Protein,肾母细胞瘤蛋白抗体Subcellular Location:
Nucleus. Nucleus, nucleolus. Cytoplasm. Note=Shuttles between nucleus and cytoplasm.
Isoform 1: Nucleus speckle.
Isoform 4: Nucleus, nucleoplasm.

Tissue Specificity:
Expressed in the kidney and a subset of hematopoietic cells.

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.
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.

Similarity:
Belongs to the EGR C2H2-type zinc-finger protein family.
Contains 4 C2H2-type zinc fingers.

Gene ID:
7490

Database links:
Entrez Gene: 7490 Human
Entrez Gene: 22431 Mouse
Entrez Gene: 24883 Rat
Omim: 607102 Human
SwissProt: P19544 Human
SwissProt: P22561 Mouse
SwissProt: P49952 Rat
Unigene: 591980 Human
Unigene: 389339 Mouse
Unigene: 92531 Rat

Wilms Tumor Protein,肾母细胞瘤蛋白抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.