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Rabbit Anti-Wilms Tumor Protein/AP Conjugated antibody (bs-6983R-AP)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-6983R-AP
英文名稱 Rabbit Anti-Wilms Tumor Protein/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標記的腎母細胞瘤蛋白抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  發育生物學  腫瘤細胞生物標志物  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat,  (predicted: Chicken, Dog, Pig, Cow, Sheep, )
產品應用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 55kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Wilms Tumor Protein
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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.

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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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