FITC标记的内脏异位相关蛋白/锌指蛋白203抗体-抗体-抗体-生物在线
FITC标记的内脏异位相关蛋白/锌指蛋白203抗体

FITC标记的内脏异位相关蛋白/锌指蛋白203抗体

商家询价

产品名称： FITC标记的内脏异位相关蛋白/锌指蛋白203抗体

英文名称： Anti-ZIC3/FITC

产品编号： HZ-11608R-FITC

产品价格： null

产品产地： 中国/上海

品牌商标： HZbscience

更新时间： 2023-08-17T10:24:20

使用范围： ICC=1:50-200 IF=1:50-200

上海沪震实业有限公司
  • 联系人 : 鲍丽雯
  • 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
  • 邮编 : 200612
  • 所在区域 : 上海
  • 电话 : 139****0749
  • 传真 : 021-60345367
  • 邮箱 : www.shzbio.net
进入展台

 Rabbit Anti-ZIC3/FITC Conjugated antibody

FITC标记的内脏异位相关蛋白/锌指蛋白203抗体

 

产品编号 bs-11608R-FITC
英文名称 Anti-ZIC3/FITC
中文名称 FITC标记的内脏异位相关蛋白/锌指蛋白203抗体
别    名 Heterotaxy 1; HTX; HTX1; ZIC 3; Zic family member 3 (odd paired Drosophila homolog heterotaxy 1); Zic family member 3; Zic3; ZIC3_HUMAN; Zinc finger protein 203; Zinc finger protein of the cerebellum 3; Zinc finger protein ZIC 3 (Zinc finger protein of the cerebellum 3); Zinc finger protein ZIC 3; ZNF203.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  发育生物学  干细胞  转录调节因子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Sheep, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 51kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ZIC3 (331-410aa)
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.
产品介绍 background:
Zic3 is a C2H2 zinc finger transcription factor that establishes a proper left-right axis and midline neural patterning during early development of the vertebrate embryo. Mutations in this gene cause X-linked visceral heterotaxy, which includes congenital heart disease and left-right axis defects in organs. Zic3 mutations in the zinc finger DNA binding domain and in the N-terminal domain result in loss of reporter gene transactivation, and mutations between amino acids 253-323 of the Zic3 protein causes aberrant cytoplasmic localization rather than the wild type nuclear localization.

Function:
Acts as transcriptional activator. Required in the earliest stages in both axial midline development and left-right (LR) asymmetry specification. Binds to the minimal GLI-consensus sequence 5'-GGGTGGTC-3'.

Subcellular Location:
Nucleus. Cytoplasm. Localizes in the cytoplasm in presence of MDFIC overexpression (By similarity). Translocation to the nucleus requires KPNA1 or KPNA6.

DISEASE:
Defects in ZIC3 are the cause of visceral heterotaxy X-linked type 1 (HTX1) [MIM:306955]. A form of visceral heterotaxy, a complex disorder due to disruption of the normal left-right asymmetry of the thoracoabdominal organs. It results in an abnormal arrangement of visceral organs, and a wide variety of congenital defects. Clinical features of visceral heterotaxy X-linked type 1 include dextrocardia, corrected transposition of great arteries, ventricular septal defect, patent ductus arteriosus, pulmonic stenosis, situs inversus viscerum, and asplenia and/or polysplenia.
Defects in ZIC3 are a cause of VACTERL association X-linked with or without hydrocephalus (VACTERLX) [MIM:314390]. A syndrome characterized by vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, renal anomalies (urethral atresia with hydronephrosis), and limb anomalies (hexadactyly, humeral hypoplasia, radial aplasia, and proximally placed thumb). Some patients may have hydrocephalus. Some cases of VACTERL-H are associated with increased chromosome breakage and rearrangement.

Similarity:
Belongs to the GLI C2H2-type zinc-finger protein family.
Contains 5 C2H2-type zinc fingers.

Database links:
UniProtKB/Swiss-Prot: O60481.1

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

ZIC3是一种C2H2锌指转录因子，在脊椎动物胚胎的早期发育过程中建立了适当的左右轴和中线神经图型。该基因的突变导致X连锁内脏异位，包括先天性心脏病和器官中的左右轴缺陷。锌指DNA结合结构域和N-末端结构域中的ZIC3突变导致报告基因反式激活的丢失，并且ZIC3蛋白的氨基酸253-323之间的突变导致异常的细胞质定位而不是野生型核定位。