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中英文說明丨艾美捷SMN酶聯(lián)免疫試劑盒

點(diǎn)擊次數(shù)：0發(fā)布時(shí)間：2023/1/17 16:49:28

中英文說明丨艾美捷SMN酶聯(lián)免疫試劑盒

更新日期：2023/1/17 16:49:28

所 在 地：中國大陸

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簡單介紹：SMN酶聯(lián)免疫試劑盒：第1個(gè)可用于神經(jīng)退行性疾病研究的現(xiàn)成SMN ELISA試劑盒。

相關(guān)標(biāo)簽：SMN酶聯(lián)免疫試劑盒 

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詳細(xì)內(nèi)容

 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 cylasm, 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.

Alternative Name: Survival motor neuron

Sensitivity: 50pg/ml (range 50-3200pg/ml)

Assay Time: 3 hours

Applications: ELISA, Colorimetric detection

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

Wavelength: 450 nm

Species reactivity: Human, Mouse

Crossreactivity: No cross reactivity.

Use/Stability: Store all components at +4ºC, except Standard at -20ºC.

Shipping: Shipped on Blue Ice

Long Term Storage: -20°C

 

SMA以肌肉無力和萎縮、功能殘疾為特征，是嬰幼兒常見的致命遺傳病。大約每35名成年人中就有一人是SMN1突變的攜帶者。

 

ADI-900-209 SMN酶聯(lián)免疫試劑盒是一種比色免疫測定試劑盒，可在3小時(shí)內(nèi)獲得結(jié)果。它是第1個(gè)可用于神經(jīng)退行性疾病研究的現(xiàn)成SMN ELISA試劑盒。

 

艾美捷ADI-900-209 SMN酶聯(lián)免疫試劑盒化學(xué)性質(zhì)：

備選名稱：存活運(yùn)動(dòng)神經(jīng)元

靈敏度：50pg/ml（范圍50-3200pg/ml）

化驗(yàn)時(shí)間：3小時(shí)

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(chǔ)存在+4ºC，標(biāo)準(zhǔn)組件除外，儲(chǔ)存在-20ºC。

運(yùn)輸：藍(lán)冰運(yùn)輸

長期儲(chǔ)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：

1.HDAC抑制劑SAHA和Dacinostat對(duì)SMA患者成纖維細(xì)胞SMN2表達(dá)影響的轉(zhuǎn)錄、甲基化和分子對(duì)接分析：J.Mohseni等人；，應(yīng)用：人類細(xì)胞中測定的SMN蛋白，；

2.丙戊酸在成人中的臨床試驗(yàn)中對(duì)血液樣本進(jìn)行的脊髓肌萎縮生物標(biāo)志物測量：S.R.Renusch等人。；J、 神經(jīng)肌肉。，應(yīng)用：使用PBMC細(xì)胞裂解物的ELISA；

3.系統(tǒng)性、癥狀后反義寡核苷酸挽救II/III型脊髓性肌萎縮癥新小鼠模型中的運(yùn)動(dòng)單位成熟延遲：L.P.Bogdanik等人。應(yīng)用：ELISA評(píng)估小鼠組織勻漿中的SMN水平；

 

來源：https://www.amyjet.com/products/ADI-900-209.shtml

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