产品货号 :  mlR23900

英文名称 :  FXR1

中文名称 :  脆性X相关蛋白1/脆性X智力低下综合征相关蛋白1抗体

别    名 :  Fragile X mental retardation syndrome related protein 1; FXR1; hFXR1p; 1110050J02Rik; 9530073J07Rik; AA959924; AI851072; FXR1H; FXR1P.  

研究领域 :  细胞生物  免疫学  染色质和核信号  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, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep,

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  62-74kDa

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human FXR1 :251-350/621

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.

PubMed :  PubMed

产品介绍 : The protein encoded by this gene is an RNA binding protein that interacts with the functionally-similar proteins FMR1 and FXR2. These proteins shuttle between the nucleus and cytoplasm and associate with polyribosomes, predominantly with the 60S ribosomal subunit. Three transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq]

Subcellular Location:

Cytoplasm.

Tissue Specificity:

Expressed in all tissues examined including heart, brain, kidney and testis.

Similarity:

Expressed in all tissues examined including heart, brain, kidney and testis. Belongs to the FMR1 family.

Contains 2 Agenet-like domains.

Contains 2 KH domains.

SWISS:

P51114

Gene ID:

8087

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。

产品图片