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Merck
CN

MABE230

抗SMN2抗体,克隆SMN-KH

clone SMN-KH, from mouse

别名:

Survival motor neuron protein, Component of gems 1, Gemin-1

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关于此项目

UNSPSC Code:
12352203
NACRES:
NA.41
eCl@ss:
32160702
Conjugate:
unconjugated
Clone:
SMN-KH, monoclonal
Application:
IHC, WB
Citations:
4
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biological source

mouse

Quality Level

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

SMN-KH, monoclonal

species reactivity

human

technique(s)

immunohistochemistry: suitable, western blot: suitable

isotype

IgG1κ

NCBI accession no.

UniProt accession no.

shipped in

wet ice

target post-translational modification

unmodified

Gene Information

human ... SMN2(6607)

General description

可观察到约35 kDa的条带
运动神经元存活蛋白 (SMN) 由两个相连的旁系同源基因SMN1和SMN2表达。SMN主要位于所有细胞的细胞质和核小体中,被认为介导剪接体小核核糖核蛋白颗粒 (snRNP) 的组装。它们还参与snoRNP的代谢,是细胞核内前体mRNA 剪接所必需的。脊髓前角功能性 SMN 的渐进性损失是脊髓性肌萎缩症 (SMA)(一种常染色体隐性遗传神经肌肉疾病)的关键病因。目前确知的儿童SMA类型有三种,成人SMA有一种。

Immunogen

对应于人SMN2的MBP标记重组蛋白。

Application

抗SMN2抗体(克隆SMN-KH)是一种用于检测SMN2(又称运动神经元存活蛋白)的小鼠单克隆抗体,&目前已通过WB & IHC应用验证。
蛋白质印迹分析:来自独立实验室的代表性批次在HeLa细胞裂解液中检测到 SMN2。

免疫组化分析:来自独立实验室的代表性批次在III 型SMA小鼠模型胸脊髓组织中检测到人SMN2。(Hua, Y., et al. (2010).Genes Dev. 24(15):1634-1644.)

Physical form

形式:纯化

Analysis Note

通过蛋白质印迹法在HeLa细胞裂解液中进行了评估。

蛋白质印迹分析:0.5 µg/mL该抗体可在10 µg HeLa细胞裂解液中检测到SMN2。

Other Notes

浓度:请参考批次特异性浓缩物的检验报告。


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存储类别

12 - Non Combustible Liquids

wgk

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable



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相关内容

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Suzan M Hammond et al.
JCI insight, 7(24) (2022-11-09)
Antisense oligonucleotides (ASOs) have emerged as one of the most innovative new genetic drug modalities. However, their high molecular weight limits their bioavailability for otherwise-treatable neurological disorders. We investigated conjugation of ASOs to an antibody against the murine transferrin receptor
Anton J Blatnik et al.
Human molecular genetics, 29(21), 3477-3492 (2020-10-20)
Spinal muscular atrophy (SMA) is caused by mutation or deletion of survival motor neuron 1 (SMN1) and retention of SMN2 leading to SMN protein deficiency. We developed an immortalized mouse embryonic fibroblast (iMEF) line in which full-length wild-type Smn (flwt-Smn)
Matthew D Howell et al.
Molecular therapy : the journal of the American Society of Gene Therapy, 25(6), 1328-1341 (2017-04-17)
Spinal muscular atrophy (SMA), the leading genetic disease of children, is caused by low levels of survival motor neuron (SMN) protein. Here, we employ A15/283, an antisense oligonucleotide targeting a deep intronic sequence/structure, to examine the impact of restoration of



全球贸易项目编号

货号GTIN
MABE23004053252437250