| 產(chǎn)品編號 | bs-23900R-BF594 |
| 英文名稱1 | Rabbit Anti-FXR1 /BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的脆性X相關(guān)蛋白1抗體 |
| 別 名 | Fragile X mental retardation syndrome related protein 1; FXR1; hFXR1p; 1110050J02Rik; 9530073J07Rik; AA959924; AI851072; FXR1H; FXR1P. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 染色質(zhì)和核信號 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) |
| 產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 62-74kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FXR1 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4. |
| 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: 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. Database links: Entrez Gene: 8087 Human Entrez Gene: 14359 Mouse Omim: 600819 Human SwissProt: P51114 Human SwissProt: Q61584 Mouse Unigene: 478407 Human Unigene: 259021 Mouse Unigene: 40468 Rat 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，又稱馬丁－貝爾綜合癥，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。 |
