产品中心
当前位置：首页>产品中心Anti-AIPL1/PE-Cy7
货号： bs-12452R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-12452R-PE-Cy7
- 英文名称
- Anti-AIPL1/PE-Cy7
- 中文名称
- PE-Cy7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.