Anti-AIPL1/FITC Conjugated抗体
产品名称： Anti-AIPL1/FITC Conjugated抗体
英文名称： Anti-AIPL1/FITC
产品编号： YB--12452R-FITC
产品价格： null
产品产地： 中国/美国
品牌商标： Ybscience
更新时间： 2023-08-17T10:29:50
使用范围： 科研使用
上海钰博生物科技有限公司
- 联系人 : 陈环环
- 地址 : 上海市沪闵路6088号龙之梦大厦8楼806室
- 邮编 : 200612
- 所在区域 : 上海
- 电话 : 183****2235
- 传真 : 021-60514606
- 邮箱 : shybio@126.com
Anti-AIPL1/FITC Conjugated抗体
| 产品编号 | YB-12452R-FITC |
| 英文名称 | Anti-AIPL1/FITC |
| 中文名称 | FITC标记的遗传性失明相关蛋白AIPL1抗体 |
| 别 名 | A930007I01Rik; Aipl1; AIPL1_HUMAN; AIPL2; Aryl hydrocarbon interacting protein like 1; Aryl hydrocarbon receptor interacting protein like 1; Aryl-hydrocarbon-interacting protein-like 1; LCA4; MGC25485; OTTHUMP00000128207; OTTMUSP00000006382; RP23-401C17.1. |
| 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 神经生物学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, 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 | 44kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human AIPL1 |
| 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: The inherited blindness associated protein, aryl hydrocarbon receptor interacting protein-like 1 (AIPL1), interacts with the cell cycle regulator protein NUB1. AIPL1 is crucial for protein folding and stabilization, as well as for protein trafficking. It localizes to the nucleus or cytoplasm and is highly expressed in the pineal gland and the retina. In the retina, AIPL1 is expressed in both developing cone and rod photoreceptors, but it is restricted to rod photoreceptors in the adult human retina. Defects in the gene encoding for AIPL1 can cause Leber congenital amaurosis type IV, an early-onset, inherited autosomal recessive disorder that results in childhood blindness. Function: May be important in protein trafficking and/or protein folding and stabilization. Subunit: Interacts with NUB1. Subcellular Location: Cytoplasm. Nucleus. Tissue Specificity: Highly expressed in retina. Specifically localized to the developing photoreceptor layer and within the photoreceptors of the adult retina. DISEASE: Defects in AIPL1 are the cause of Leber congenital amaurosis type 4 (LCA4) [MIM:604393]. LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children. Similarity: Contains 1 PPIase FKBP-type domain. Contains 3 TPR repeats. Database links: Entrez Gene: 23746 Human Entrez Gene: 114230 Mouse Entrez Gene: 59110 Rat Omim: 604392 Human SwissProt: Q9NZN9 Human SwissProt: Q924K1 Mouse SwissProt: Q9JLG9 Rat Unigene: 279887 Human Unigene: 95707 Mouse Unigene: 102037 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |