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    Rabbit Anti-FXR1 /PE-Cy5.5 Conjugated antibody (bs-23900R-PE-Cy5.5)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-23900R-PE-Cy5.5
    英文名稱(chēng) Rabbit Anti-FXR1 /PE-Cy5.5 Conjugated antibody
    中文名稱(chēng) PE-Cy5.5標記的脆性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.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul  
    研究領(lǐng)域 細胞生物  免疫學(xué)  染色質(zhì)和核信號  神經(jīng)生物學(xué)  信號轉導  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
    產(chǎn)品應用 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, 0.05M PB, pH 7.5.
    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

    Entrez Gene: 361927 Rat

    Omim: 600819 Human

    SwissProt: P51114 Human

    SwissProt: Q61584 Mouse

    SwissProt: Q5XI81 Rat

    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(chēng)馬?。悹柧C合癥,是一種遺傳疾病。該綜合癥可以導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(zhù)X染色體上一個(gè)簡(jiǎn)單的三核苷酸基因序列(CGG)的擴增。這種擴增導致了一種稱(chēng)為FMR-1的蛋白質(zhì)無(wú)法在病人體內表達,而該蛋白質(zhì)是神經(jīng)的正常發(fā)育必不可少的。
    根據CGG重復序列的長(cháng)度,目前普遍認可將脆性X綜合癥分為四種類(lèi)型:正常人(含有19-31個(gè)CGG重復序列),前突變者(含有55-200個(gè)CGG重復序列),全突變者(含有200個(gè)以上的CGG重復序列),過(guò)渡型,又稱(chēng)“灰色區域型”(含有40-60個(gè)重復)。脆性X綜合征這是一種導致智力低下的遺傳疾病,是導致人群中智力低下的第二大病因——僅次于21三體綜合癥。
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