| 產(chǎn)品編號(hào) | bs-6975R-BF555 |
| 英文名稱 | Rabbit Anti-FXR2/BF555 Conjugated antibody |
| 中文名稱 | BF555標(biāo)記的脆性X相關(guān)蛋白樣2抗體 |
| 別 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. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書 | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 神經(jīng)生物學(xué) 表觀遺傳學(xué) |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, ) |
| 產(chǎn)品應(yīng)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 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FXR2 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
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: 100343838 Rabbit 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綜合癥，又稱馬?。悹柧C合癥，是一種遺傳疾病。該綜合癥可以導(dǎo)致一系列的特征性癥狀，包括生理、智力、情緒、以及行為上的異常。癥狀的輕重各有不同。該疾病伴隨著X染色體上一個(gè)簡(jiǎn)單的三核苷酸基因序列（CGG）的擴(kuò)增。這種擴(kuò)增導(dǎo)致了一種稱為FMR-1的蛋白質(zhì)無(wú)法在病人體內(nèi)表達(dá)，而該蛋白質(zhì)是神經(jīng)的正常發(fā)育必不可少的。 根據(jù)CGG重復(fù)序列的長(zhǎng)度，目前普遍認(rèn)可將脆性X綜合癥分為四種類型：正常人（含有19－31個(gè)CGG重復(fù)序列），前突變者（含有55－200個(gè)CGG重復(fù)序列），全突變者（含有200個(gè)以上的CGG重復(fù)序列），過(guò)渡型，又稱“灰色區(qū)域型”（含有40－60個(gè)重復(fù)）。脆性X綜合征這是一種導(dǎo)致智力低下的遺傳疾病，是導(dǎo)致人群中智力低下的第二大病因——僅次于21三體綜合癥。 |
