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| 中文名稱 | 內臟異位相關蛋白/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.?? |
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| 研究領域 | 細胞生物??發育生物學??干細胞??轉錄調節因子?? |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應 | Mouse,?Rat,? (predicted: Human,?Chicken,?Dog,?Pig,?Cow,?Sheep,?) |
| 產品應用 | WB=1:500-2000?ELISA=1:500-1000?IHC-P=1:100-500?IHC-F=1:100-500?ICC=1:100-500?IF=1:100-500?（石蠟切片需做抗原修復） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分?子?量 | 51kDa |
| 細胞定位 | 細胞核?細胞漿? |
| 性????狀 | Liquid |
| 濃????度 | 1mg/ml |
| 免?疫?原 | KLH conjugated synthetic peptide derived from human ZIC3 (331-410aa):331-410/467? |
| 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 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產品介紹 | 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. SWISS: O60481 Gene ID: 7547 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. ? |
| 產品圖片 |  Sample:Cerebellum (Rat) Lysate at 40 ug Primary: Anti-ZIC3 ? (bs-11608R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 51 kD Observed band size: 49/50 kD  Sample:Cerebrum (Mouse) Lysate at 40 ug Cerebellum (Mouse) Lysate at 40 ug Embryo (Mouse) Lysate at 40 ug Primary: Anti-ZIC3 ? (bs-11608R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 51 kD Observed band size: 49/50 kD  Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ZIC3) Polyclonal Antibody, Unconjugated (bs-11608R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ZIC3) Polyclonal Antibody, Unconjugated (bs-11608R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining. |
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