产品名称
抗SMN2抗体，克隆SMN-KH, clone SMN-KH, from mouse
biological source
mouse
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
Quality Level
Gene Information
human ... SMN2(6607)
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。
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。
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.)
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.)
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。
Other Notes
浓度：请参考批次特异性浓缩物的检验报告。
Physical form
形式：纯化
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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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JCI insight, 7(24) (2022-11-09)
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EMBO molecular medicine, 15(11), e17683-e17683 (2023-09-19)
Spinal muscular atrophy (SMA) is a leading genetic cause of infant mortality. The advent of approved treatments for this devastating condition has significantly changed SMA patients' life expectancy and quality of life. Nevertheless, these are not without limitations, and research
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
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