ADI-900-209?SMN酶聯(lián)免疫試劑盒：

高度靈敏的測量，可檢測低至50 pg/mL的SMN

超過半定量蛋白質(zhì)印跡分析的全定量結(jié)果

方便易用的液體色碼試劑和預(yù)涂96孔板，可節(jié)省時間并將誤差降至最低

高吞吐量格式，3小時內(nèi)可處理多達(dá)39份樣本，一式兩份

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Survival Motor Neuron (SMN) is a ~38 kDa protein produced chiefly by the SMN1 gene, located on the telomeric portion of chromosome 5q. A nearly identical centromeric copy of the gene (SMN2) also produces a small amount of full-length SMN protein, but due to a translationally silent C-T transition that results in alternative splicing of the pre-mRNA, most of the resulting SMN is truncated, causing reduced protein stability and lower overall SMN levels. Deletion or mutation of the SMN1 gene results in a reduced level of full-length SMN protein and manifests as a range of neuromuscular phenotypes in humans as the disease spinal muscular atrophy (SMA). SMA is characterized by muscle weakness and atrophy, functional disability and is the most common lethal genetic disease of infants and toddlers. Approximately one in 35 adults is a ?carrier of the SMN1 mutation. The incidence of SMA is 1 in 6,000 to 1 in 10,000 live births. SMN protein is present in the cell cytoplasm, and also in the nucleus where it is concentrated in?“gem”?structures associated with Cajal bodies. SMN protein is a constituent of Gemin-containing complexes, and is thought to participate in many aspects of RNA metabolism. SMN complexes have been shown to mediate the assembly of uridine rich small nuclear ribonucleoproteins (snRNPs), which in turn act as critical components of spliceosomes.

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生存運(yùn)動神經(jīng)元（SMN）是一種~38kDa的蛋白質(zhì)，主要由SMN1基因產(chǎn)生，位于染色體5q的端粒部分。該基因的幾乎相同的著絲?？截悾⊿MN2）也產(chǎn)生少量的全長SMN蛋白，但由于翻譯沉默的C-T轉(zhuǎn)換導(dǎo)致前mRNA的選擇性剪接，大多數(shù)產(chǎn)生的SMN被截斷，導(dǎo)致蛋白穩(wěn)定性降低，SMN總水平降低。SMN1基因的缺失或突變導(dǎo)致全長SMN蛋白水平降低，并在人類中表現(xiàn)為一系列神經(jīng)肌肉表型，如脊髓性肌萎縮癥（SMA）。SMA以肌肉無力和萎縮、功能殘疾為特征，是嬰幼兒最常見的致命遺傳病。

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艾美捷ADI-900-209?SMNSMN酶聯(lián)免疫試劑盒：

備選名稱：存活運(yùn)動神經(jīng)元

靈敏度：50pg/ml（范圍50-3200pg/ml）

化驗時間：3小時

應(yīng)用：ELISA，比色檢測

應(yīng)用說明：用于定量測定細(xì)胞裂解物樣品中的人和小鼠SMN。

波長：450 nm

物種反應(yīng)性：人、小鼠

交叉反應(yīng)性：無交叉反應(yīng)性。

使用/穩(wěn)定性：將所有組件儲存在+4oC，標(biāo)準(zhǔn)組件除外，儲存在-20oC。

運(yùn)輸：藍(lán)冰運(yùn)輸

長期儲存：-20°C

內(nèi)容：微量滴定板、綴合物、抗體、測定緩沖液13、洗滌緩沖液濃縮液、標(biāo)準(zhǔn)品、TMB底物、停止溶液2、提取試劑4

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SMN酶聯(lián)免疫試劑盒文獻(xiàn)參考：

一種通過成像流式細(xì)胞術(shù)將存活運(yùn)動神經(jīng)元蛋白作為脊髓肌萎縮的生物標(biāo)志物的新評估方法：M.Arakawa等人。；生物化學(xué)。生物物理學(xué)。Res.Commun公司。453368（2014），應(yīng)用：在皮膚成纖維細(xì)胞上的應(yīng)用；

嚴(yán)重的SMA小鼠表現(xiàn)出不能通過HDACi JNJ-26481585:J.Schreml等人的治療來挽救的器官損傷。；歐洲人類遺傳學(xué)雜志。21，643（2013），應(yīng)用：使用SMA成纖維細(xì)胞系的EIA;

評估外周血單核細(xì)胞處理和生存運(yùn)動神經(jīng)元蛋白分析：D.T.Kobayashi等人。；PLoS One 7，e50763（2012），應(yīng)用：使用人細(xì)胞裂解物的ELISA;

脊髓性肌萎縮癥（BforSMA）臨床研究生物標(biāo)志物中SMN蛋白、轉(zhuǎn)錄物和拷貝數(shù)的評估：T.O.Crawford等人。；PLoS One 7，e33572（2012）;

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