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SMN ELISA kit——ENZO热销产品

2023-09-01
浏览次数: 25

首个商业化的可用于神经退行性疾病研究的即用型SMN ELISA试剂盒

 

运动神经元生存蛋白(SMN)是一种约38kDa的蛋白质,主要由位于5q染色体端粒部分的SMN1基因产生。几乎相同的着丝粒拷贝(SMN2)也产生少量的全长SMN蛋白,但由于翻译沉默的C-T转换导致mRNA前体的选择性剪接,所产生的大部分SMN被截断,导致蛋白稳定性降低和整体SMN水平下降。SMN1基因的缺失或突变导致全长SMN蛋白水平降低,在人类中表现为一系列的神经肌肉表型,即脊髓性肌肉萎缩症(SMA)疾病。SMA的特点是肌肉无力、萎缩与功能障碍,是婴幼儿最常见的致命遗传病。大约35个成年人中就有一个是SMN1突变的携带者。活产婴中SMA的发病率为1/6,000至1/10,000。SMN蛋白存在于细胞质中,也存在于细胞核中,它集中在与Cajal体相关的“宝石”结构中。SMN蛋白是含有Gemin的复合物的成分,被认为参与了RNA代谢的许多方面。SMN复合物已被证明可介导富含尿苷的小核糖核酸蛋白(snRNPs)的组装,而snRNPs又充当了剪接体的关键成分。

欣博盛生物作为Enzo Life Sciences授权中国一级代理,可提供该产品,欢迎咨询。

 

产品特点

高灵敏度,可检测低至50 pg/mL的运动神经元生存蛋白

◆ 完全定量的结果,优于半定量的Western blot分析

◆ 简单易用的预包被板与颜色编码试剂,最大限度地减少实验误差

◆ 高通量,3小时内可得39份样品(含复孔)的检测结果

 

产品信息

产品货号

ADI-900-209

产品名称

SMN ELISA kit(运动神经元生存蛋白ELISA试剂盒)

规格

96 wells

灵敏度

50 pg/ml

检测范围

50-3200 pg/ml

其他名称

Survival motor neuron

应用

ELISA, Colorimetric detection

适用样本类型

For the quantitative determination of human and mouse SMN in cell lysate samples.

种属

Human, Mouse

检测波长

450 nm

使用/稳定性

Store all components at +4ºC, except Standard at -20ºC

运输温度

Dry Ice and Blue Ice

长期保存温度

-20°C

试剂盒组分

Microtiter Plate, Conjugate, Antibody, Assay Buffer 13, Wash Buffer Concentrate, 

Standard, TMB Substrate, Stop Solution 2, Extraction Reagent 4

 

标准曲线示例

SMN ELISA kit——ENZO热销产品

 

SMN ELISA kit——ENZO热销产品 

  

部分产品引用文献

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)

 

 

 

SMN ELISA kit——ENZO热销产品

详情请咨询 ENZO 金牌代理商-欣博盛生物科技

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