肾母细胞瘤X蛋白抗体
规格：1mg/1ml
英文名： FAM123B
别名： AMER1; FAM 123B; Family with sequence similarity 123B; FLJ39827; OSCS; Protein FAM123B; RP11 403E24.2; Wilms tumor gene on the X chromosome protein; Wilms tumor on the X; WTX; AMER1_HUMAN.
分子量： 124kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human FAM123B/
交叉反应：Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞核 细胞浆 细胞膜
肾母细胞瘤X蛋白抗体产品介绍：background: The protein encoded by this gene upregulates trancriptional activation by the Wilms tumor protein and interacts with many other proteins, including CTNNB1, APC, AXIN1, and AXIN2. Defects in this gene are a cause of osteopathia striata with cranial sclerosis (OSCS). Function: Regulator of the canonical Wnt signaling pathway. Acts by specifically binding phosphatidylinositol 4,5-bisphosphate (PtdIns(4,5)P2), translocating to the cell membrane and interacting with key regulators of the canonical Wnt signaling pathway, such as components of the beta-catenin destruction complex. Acts both as a positive and negative regulator of the Wnt signaling pathway, depending on the context: acts as a positive regulator by promoting LRP6 phosphorylation. Also acts as a negative regulator by acting as a scaffold protein for the beta-catenin destruction complex and promoting stabilization of Axin at the cell membrane. Promotes CTNNB1 ubiquitination and degradation. Involved in kidney development. Subunit: Interacts with CTNNB1, AXIN1, LRP6, KEAP1, APC and BTRC. Interacts with 肾母细胞瘤X蛋白抗体SCF (SKP1-CUL1-F-box protein) E3 ubiquitin-protein ligase complexes containing BTRC and/or FBXW11. Identified in the beta-catenin destruction complex containing CTNNB1, APC, AXIN1 and AXIN2. Interacts with WT1. Subcellular Location: Cytoplasm. Cell membrane; Peripheral membrane protein; Cytoplasmic side. Nucleus. Note=Shuttles between nucleus and cytoplasm. Detected in nuclear paraspeckles that are found close to splicing speckles. Translocates to the cell membrane following binding to PtdIns(4,5)P2. DISEASE: Defects in FAM123B are the cause of osteopathia striata with cranial sclerosis (OSCS) [MIM:300373]. OSCS is an X-linked dominant sclerosing bone dysplasia that presents in females with macrocephaly肾母细胞瘤X蛋白抗体, cleft palate, mild learning disabilities, sclerosis of the long bones and skull, and longitudinal striations visible on radiographs of the long bones, pelvis, and scapulae. In males this entity is usually associated with fetal or neonatal lethality. Similarity: Belongs to the Amer family. Database links: UniProtKB/Swiss-Prot: Q5JTC6.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
肾母细胞瘤X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=1:100-500 （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.
研究领域：肿瘤 细胞生物 信号转导 干细胞
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid