ZIC3 Antibody
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中文名稱：ZIC3兔多克隆抗體
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貨號：CSB-PA026486ESR2HU
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規格：￥440
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圖片：
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Immunohistochemistry of paraffin-embedded human melanoma using CSB-PA026486ESR2HU at dilution of 1:100
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) ZIC3 Polyclonal antibody
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Uniprot No.：
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基因名：
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別名：Heterotaxy 1 antibody; HTX antibody; HTX1 antibody; VACTERLX antibody; ZIC 3 antibody; Zic family member 3 (odd paired Drosophila homolog heterotaxy 1) antibody; Zic family member 3 antibody; Zic3 antibody; ZIC3_HUMAN antibody; Zinc finger protein 203 antibody; Zinc finger protein of the cerebellum 3 antibody; Zinc finger protein ZIC 3 (Zinc finger protein of the cerebellum 3) antibody; Zinc finger protein ZIC 3 antibody; ZNF203 antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human Zinc finger protein ZIC 3 protein (1-240AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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產品提供形式：Liquid
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應用范圍：ELISA, IHC
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推薦稀釋比：
Application Recommended Dilution IHC 1:20-1:200 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：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'.
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基因功能參考文獻：
- current understanding of ZIC3 function and describe the potential role ZIC3 plays in important signalling pathways and their links to heterotaxy. PMID: 29442328
- Six novel pathogenic variants were identified in either male patients with heterotaxy or a female patient with multiple male deaths due to heterotaxy in the family. Truncating variants showed abnormal trafficking of mutated ZIC3 proteins. PMID: 27406248
- Targeted Resequencing of 29 Candidate Genes and Mouse Expression Studies Implicate ZIC3 and FOXF1 in Human VATER/VACTERL Association PMID: 26294094
- detected the expression level of miR-564 and ZIC3 protein in tissue specimens, and found a significant negative correlation between them. Patients with low levels of miR-564 showed a poorer overall survival PMID: 26498524
- ZIC3 sequencing from unrelated patients with heterotaxy and congenital heart disease identified variants in 5.2% of sporadic male cases some of which were novel. Functional analyses show aberrant cytoplasmic localization. PMID: 24123890
- sumoylation targets human ZIC3 primarily on the consensus lysine residue K248, which is critical for the nuclear retention of ZIC3. PMID: 23872418
- Case Reports: situs inversus totalis and X-linked heterotaxy as a result of novel ZIC3 mutation. PMID: 22171628
- ZIC3 mutations are an important etiology in sporadic and familial heterotaxy. PMID: 23427188
- Mutations in Zinc Finger Protein of the Cerebellum 3 were identified in 4 of the 47 patients (8.5%) with heterotaxy syndrome. Our results expand the mutation spectrum of monogenic heterotaxy syndrome with associated cardiac anomalies. PMID: 21864452
- Data show that transcript, termed Zic3-B, encompasses exons 1, 2, and 4 whereas Zic3-A encompasses exons 1, 2, and 3. PMID: 21858219
- Disruption of Gli3-Zic3 interaction in the critical period for ventral body wall formation may contribute to omphalocele phenotype in Cd chick model. PMID: 21069353
- we have focused on the regulation of the Zic3 gene, which codes for a zinc finger transcription factor expressed in the organizer region at the beginning of gastrulation PMID: 12963115
- ZIC3 mutations in three classic heterotaxy kindreds and two sporadic congenital heart defect cases PMID: 14681828
- Results suggest that Zic3 plays a role in intra-retinal axon targeting, possibly through regulation of the expression of specific downstream genes involved in axon guidance. PMID: 14985256
- A positional effect caused by the balanced (X;21) translocation may be responsible for functional nullisomy of ZIC3 PMID: 15470371
- Nuclear import and export signals are essential for proper cellular trafficking and function of ZIC3. PMID: 17185387
- We studied a series of 42 cases of transposition of the great arteries; mutation in the X chromosome at the ZIC3 gene was found in two affected siblings (one male, one female) and their unaffected mother PMID: 17295247
- ZIC3 has a role in regulating cardiac gene expression PMID: 17468179
- in vitro interactions of ZIC3 with GLI3 and the effect of ZIC3 mutations identified in patients with either heterotaxy or isolated cardiovascular malformations. PMID: 17764085
- results indicate that ZIC3 is imported into the cell nucleus by the Karyopherin (Importin) system and that the impaired nuclear localization by the ZF1 mutation is not due to a direct influence on the nuclear localization signal PMID: 18716025
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相關疾病：Heterotaxy, visceral, 1, X-linked (HTX1); VACTERL association X-linked with or without hydrocephalus (VACTERLX); Congenital heart defects, multiple types, 1, X-linked (CHTD1)
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亞細胞定位：Nucleus. Cytoplasm.
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蛋白家族：GLI C2H2-type zinc-finger protein family
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數據庫鏈接：
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