FITC标记的叉头相关转录因子3/FOXC1抗体
产品名称： FITC标记的叉头相关转录因子3/FOXC1抗体
英文名称： Anti-FREAC3/FITC
产品编号： HZ-6642R-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： Flow-Cyt=1:50-200 IF=1:50-200
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Rabbit Anti-FREAC3/FITC Conjugated antibody
FITC标记的叉头相关转录因子3/FOXC1抗体
| 英文名称 | Anti-FREAC3/FITC |
| 中文名称 | FITC标记的叉头相关转录因子3/FOXC1抗体 |
| 别 名 | ARA; FKH L7; FKHL 7; FKHL7; Forkhead (Drosophila) like 7; Forkhead; forkhead box C1; Forkhead box protein C1; Forkhead drosophila homolog like 7; Forkhead like 7; Forkhead related activator 3; Forkhead related protein FKHL7; Forkhead related transcription factor 3; Forkhead-related protein FKHL7; Forkhead-related transcription factor 3; FOX C1; FOXC 1; FOXC1; FOXC1_HUMAN; FREAC 3; FREAC-3; FREAC3; homolog-like 7; IGDA; IHG 1; IHG1; IRID 1; IRID1; Iridogoniodysgenesis type 1; Myeloid factor delta. |
| 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 转录调节因子 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Cow, Horse, |
| 产品应用 | Flow-Cyt=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 | 57kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FOXC1/FREAC3 |
| 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: Binding of FREAC-3 and FREAC-4 to their cognate sites results in bending of the DNA at an angle of 80-90 degrees. Involvement in disease; Defects in FOXC1 are the cause of Axenfeld-Rieger syndrome type 3 (RIEG3); also known as Axenfeld-Rieger syndrome (ARS) or Axenfeld syndrome or Axenfeld anomaly. It is characterized by posterior corneal embryotoxon, prominent Schwalbe line and iris adhesion to the Schwalbe line. Other features may be hypertelorism (wide spacing of the eyes), hypoplasia of the malar bones, congenital absence of some teeth and mental retardation. When associated with tooth anomalies, the disorder is known as Rieger syndrome. Glaucoma is a progressive blinding condition that occurs in approximately half of patients with Axenfeld-Rieger malformations. Function: Binding of FREAC-3 and FREAC-4 to their cognate sites results in bending of the DNA at an angle of 80-90 degrees. Subunit: Monomer. Subcellular Location: Nucleus. Tissue Specificity: Expressed in all tissues and cell lines examined. DISEASE: Defects in FOXC1 are the cause of iridogoniodysgenesis anomaly (IGDA) [MIM:601631]. IGDA is an autosomal dominant phenotype characterized by iris hypoplasia, goniodysgenesis, and juvenile glaucoma. [DISEASE] Defects in FOXC1 are a cause of Peters anomaly (PAN) [MIM:604229]. Peters anomaly consists of a central corneal leukoma, absence of the posterior corneal stroma and Descemet membrane, and a variable degree of iris and lenticular attachments to the central aspect of the posterior cornea. Similarity: Contains 1 fork-head DNA-binding domain. Database links: Entrez Gene: 2296 Human Entrez Gene: 17300 Mouse GenBank: NP_001444 Human Omim: 601090 Human SwissProt: Q12948 Human SwissProt: Q61572 Mouse SwissProt: Q32NP8 Xenopus laevis Unigene: 348883 Human Unigene: 12949 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
FRAC-3和FRAC-4与它们同源位点的结合导致DNA在80-90度的角度弯曲。
FXC1中的缺陷是AxEnFeld－RieGER综合征3型（RIGE3）的原因；也称为Axenfeld Rieger综合征（ARS）或AxEnFeld综合征或Axenfeld异常。其特征为角膜后胚胎毒素、Skavbe线、虹膜粘连等。其他特征可能是过宽（眼宽），颧骨发育不良，先天性缺牙和智力迟钝。当与牙齿异常相关时，这种疾病被称为里格综合征。青光眼是一种渐进性致盲状态，发生在大约一半的Axenfeld Rieger malformations患者中。