首個(gè)商業(yè)化的可用于神經(jīng)退行性疾病研究的即用型SMN ELISA試劑盒
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運(yùn)動(dòng)神經(jīng)元生存蛋白（SMN）是一種約38kDa的蛋白質(zhì)，主要由位于5q染色體端粒部分的SMN1基因產(chǎn)生。幾乎相同的著絲?？截悾⊿MN2）也產(chǎn)生少量的全長(zhǎng)SMN蛋白，但由于翻譯沉默的C-T轉(zhuǎn)換導(dǎo)致mRNA前體的選擇性剪接，所產(chǎn)生的大部分SMN被截?cái)啵瑢?dǎo)致蛋白穩(wěn)定性降低和整體SMN水平下降。SMN1基因的缺失或突變導(dǎo)致全長(zhǎng)SMN蛋白水平降低，在人類中表現(xiàn)為一系列的神經(jīng)肌肉表型，即脊髓性肌肉萎縮癥（SMA）疾病。SMA的特點(diǎn)是肌肉無力、萎縮與功能障礙，是嬰幼兒最常見的致命遺傳病。大約35個(gè)成年人中就有一個(gè)是SMN1突變的攜帶者。活產(chǎn)嬰中SMA的發(fā)病率為1/6,000至1/10,000。SMN蛋白存在于細(xì)胞質(zhì)中，也存在于細(xì)胞核中，它集中在與Cajal體相關(guān)的“寶石”結(jié)構(gòu)中。SMN蛋白是含有Gemin的復(fù)合物的成分，被認(rèn)為參與了RNA代謝的許多方面。SMN復(fù)合物已被證明可介導(dǎo)富含尿苷的小核糖核酸蛋白（snRNPs）的組裝，而snRNPs又充當(dāng)了剪接體的關(guān)鍵成分。
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產(chǎn)品特點(diǎn)
◆ 高靈敏度，可檢測(cè)低至50 pg/mL的運(yùn)動(dòng)神經(jīng)元生存蛋白
◆ 完全定量的結(jié)果，優(yōu)于半定量的Western blot分析
◆ 簡(jiǎn)單易用的預(yù)包被板與顏色編碼試劑，最大限度地減少實(shí)驗(yàn)誤差
◆ 高通量，3小時(shí)內(nèi)可得39份樣品（含復(fù)孔）的檢測(cè)結(jié)果
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產(chǎn)品信息
| 產(chǎn)品貨號(hào) | ADI-900-209 |
產(chǎn)品名稱 | SMN ELISA kit（運(yùn)動(dòng)神經(jīng)元生存蛋白ELISA試劑盒） |
規(guī)格 | 96 wells |
靈敏度 | 50 pg/ml |
檢測(cè)范圍 | 50-3200 pg/ml |
其他名稱 | Survival motor neuron |
應(yīng)用 | ELISA, Colorimetric detection |
適用樣本類型 | For the quantitative determination of human and mouse SMN in cell lysate samples. |
種屬 | Human, Mouse |
檢測(cè)波長(zhǎng) | 450 nm |
使用/穩(wěn)定性 | Store all components at +4oC, except Standard at -20oC |
運(yùn)輸溫度 | Dry Ice and Blue Ice |
長(zhǎng)期保存溫度 | -20°C |
試劑盒組分 | Microtiter Plate, Conjugate, Antibody, Assay Buffer 13, Wash Buffer Concentrate,? Standard, TMB Substrate, Stop Solution 2, Extraction Reagent 4 |
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標(biāo)準(zhǔn)曲線示例
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部分產(chǎn)品引用文獻(xiàn)
A Comparative Study of SMN Protein and mRNA in Blood and Fibroblasts in Patients with Spinal Muscular Atrophy and Healthy Controls: R.I. Wadman, et al.; PLoS One 11, e0167087 (2016)
Protective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy: M.E. Butchbach, et al.; Exp. Neurol. 279, 13 (2016)
SMN Protein Can Be Reliably Measured in Whole Blood with an Electrochemiluminescence (ECL) Immunoassay: Implications for Clinical Trials: P. Zaworski, et al.; PLoS One 11, e0150640 (2016)
Transcript, methylation and molecular docking analyses of the effects of HDAC inhibitors, SAHA and Dacinostat, on SMN2 expression in fibroblasts of SMA patients: J. Mohseni, et al. ; J. Hum. Genet. 61, 823 (2016)
Spinal Muscular Atrophy Biomarker Measurements from Blood Samples in a Clinical Trial of Valproic Acid in Ambulatory Adults: S.R. Renusch, et al.; J. Neuromuscul. Dis. 2, 119 (2015)
Systemic, postsymptomatic antisense oligonucleotide rescues motor unit maturation delay in a new mouse model for type II/III spinal muscular atrophy : L.P. Bogdanik, et al.; PNAS 112, E5863 (2015)
A novel evaluation method of survival motor neuron protein as a biomarker of spinal muscular atrophy by imaging flow cytometry: M. Arakawa, et al.; Biochem. Biophys. Res. Commun. 453, 368 (2014)
Severe SMA mice show organ impairment that cannot be rescued by therapy with the HDACi JNJ-26481585: J. Schreml, et al.; Eur. J. Hum. Genet. 21, 643 (2013)
Evaluation of Peripheral Blood Mononuclear Cell Processing and Analysis for Survival Motor Neuron Protein: D.T. Kobayashi, et al.; PLoS One 7, e50763 (2012)
Evaluation of SMN protein, transcript, and copy number in the biomarkers for spinal muscular atrophy (BforSMA) clinical study: T.O. Crawford, et al.; PLoS One 7, e33572 (2012)
Utility of Survival Motor Neuron ELISA for Spinal Muscular Atrophy Clinical and Preclinical Analyses: D.T. Kobayashi, et al.; PLoS One 6, e24269 (2011)
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