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Rabbit Anti-ZIC3/BF647 Conjugated antibody (bs-11608R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-11608R-BF647
英文名稱 Rabbit Anti-ZIC3/BF647 Conjugated antibody
中文名稱 BF647標記的內(nèi)臟異位相關蛋白/鋅指蛋白203抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  發(fā)育生物學  干細胞  轉錄調節(jié)因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Sheep, )
產(chǎn)品應用 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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ZIC3 (331-410aa)
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(chǎn)品介紹 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:

Entrez Gene: 7547 Human

Entrez Gene: 22773 Mouse

Entrez Gene: 367944 Rat

Omim: 300265 Human

SwissProt: O60481 Human

SwissProt: Q62521 Mouse

Unigene: 111227 Human

Unigene: 255890 Mouse



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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