脆性X相关蛋白样2抗体
规格:1mg/1ml
英文名: FXR2
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 pr
分子量: 74kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human FXR2
交叉反应:Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
细胞定位:细胞浆
脆性X相关蛋白样2抗体产品介绍: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.脆性X相关蛋白样2抗体 Gene ID: 9513 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,脆性X相关蛋白样2抗体是一种遗传**。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**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**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**,是导致人群中智力低下的**大病因——仅次于21三体综合症。
脆性X相关蛋白样2抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 (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.
研究领域:细胞生物 **学 神经生物学 表观遗传学
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid