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货号： bs-6975R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-6975R-AF555
- 英文名称
- Anti-FXR2/AF555
- 中文名称
- AF555标记的脆性X相关蛋白样2抗体
- 别 名
- FMR1L2; Fragile X mental retardation 1 like 2; Fragile X mental retardation autosomal homolog 2; Fragile X mental retardation gene autosomal homolog 2; Fragile X mental retardation syndrome related protein 2; Fragile X mental retardation syndrome-related protein 2; FXR 2; FXR2; FXR2 PEN; FXR2_HUMAN; Human fragile X mental retardation syndrome related protein FXR2 mRNA complete cds.
- 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 免疫学 神经生物学 表观遗传学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 74kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human FXR2
- 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:
Fragile X syndrome is caused by the absence of the fragile X mental-retardation protein (FMRP). FMRP is the archetype of a class of cytoplasmic mRNA-binding proteins that includes the fragile X-related 1 and 2 proteins (FXR1 and FXR2). The fragile X-related proteins FXR1 and FXR2 contain a functional nucleolar-targeting signal equivalent to the HIV-1 regulatory proteins.
Function:
RNA-binding protein.
Subunit:
Interacts with FMR1 and FXR1. Interacts with CYFIP2 but not with CYFIP1. Interacts with TDRD3.
Subcellular Location:
Cytoplasm.
Similarity:
Belongs to the FMR1 family.
Contains 2 Agenet-like domains.
Contains 2 KH domains.
Database links:Entrez Gene: 9513 Human
Entrez Gene: 100512131 Pig
Entrez Gene: 100343838 Rabbit
Entrez Gene: 287433 Rat
Omim: 605339 Human
SwissProt: P51116 Human
Unigene: 52788 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
脆性X综合症，又称马丁－贝尔综合症，是一种遗传疾病。该综合症可以导致一系列的特征性症状，包括生理、智力、情绪、以及行为上的异常。症状的轻重各有不同。该疾病伴随着X染色体上一个简单的三核苷酸基因序列（CGG）的扩增。这种扩增导致了一种称为FMR-1的蛋白质无法在病人体内表达，而该蛋白质是神经的正常发育必不可少的。
根据CGG重复序列的长度，目前普遍认可将脆性X综合症分为四种类型：正常人（含有19－31个CGG重复序列），前突变者（含有55－200个CGG重复序列），全突变者（含有200个以上的CGG重复序列），过渡型，又称“灰色区域型”（含有40－60个重复）。脆性X综合征这是一种导致智力低下的遗传疾病，是导致人群中智力低下的第二大病因——仅次于21三体综合症。