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Rabbit Anti-FXR1  antibody (bs-23900R)  
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產(chǎn)品編號(hào) bs-23900R
英文名稱 Rabbit Anti-FXR1  antibody
中文名稱 脆性X相關(guān)蛋白1/脆性X智力低下綜合征相關(guān)蛋白1抗體
別    名 Fragile X mental retardation syndrome related protein 1; FXR1; hFXR1p; 1110050J02Rik; 9530073J07Rik; AA959924; AI851072; FXR1H; FXR1P.  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  染色質(zhì)和核信號(hào)  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human (predicted: Mouse,Rat,Rabbit,Pig,Sheep,Cow,Chicken,Dog,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FXR1 : 251-350/621 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
P51114

Gene ID:
8087

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



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。
產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (Human kidney tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (FXR1) Polyclonal Antibody, Unconjugated (bs-23900R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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