别 名 Fragile X mental retardation syndrome related protein 1; FXR1; hFXR1p; 1110050J02Rik; 9530073J07Rik; AA959924; AI851072; FXR1H; FXR1P.
3)纯化的抗体溶液应以较高的浓度(如lmg/m1)在中性pH下保存。:常用的抗体储存浓度高达l0mg/ml。较低浓度的抗体冻存前应浓缩。所有标准的浓缩方法(如超滤法),皆可使用。还有一个简单的方法是用蛋白A或蛋白G亲和柱来浓缩溶液。如果纯化的抗体不是用于标记,可将它们以较低浓度储存于加有1%BSA的溶液中。
4)经纯化制备的抗体在常用的缓冲液中是稳定的。其DH应保持在中性左右。如果pH在7-8之间,即使保存多年,对抗体也无损害。多数情况下,盐浓度适于保持在0-150mmol/L之间,但在长期存放的抗体中,盐溶液浓度高达500mmol/L时,对
脆性X相关蛋白1抗体可能有损害。如果没有其他说明.律议用PBS或50mmol/LTris(DH8.0)溶液长期存放抗体。
抗体来源 Rabbit
克隆类型 polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Rabbit
产品类型 一抗
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
蛋白分子量 predicted molecular weight: 62-74kDa
性 状 Lyophilized or Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human FXR1
亚 型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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.
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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
脆性X相关蛋白1抗体产品介绍 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.
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。