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Merck

S2944

Sigma-Aldrich

Anti-SMN antibody, Mouse monoclonal

clone 2B1, purified from hybridoma cell culture

別名:

Anti-Survival of Motor Neurons

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About This Item

MDL番号:
UNSPSCコード:
12352203
NACRES:
NA.41

由来生物

mouse

品質水準

結合体

unconjugated

抗体製品の状態

purified from hybridoma cell culture

抗体製品タイプ

primary antibodies

クローン

2B1, monoclonal

形状

buffered aqueous solution

化学種の反応性

Xenopus, human, mouse

濃度

~2 mg/mL

テクニック

microarray: suitable
western blot: 2-4 μg/mL using A431 cell extract

アイソタイプ

IgG1

輸送温度

dry ice

保管温度

−20°C

ターゲットの翻訳後修飾

unmodified

遺伝子情報

詳細

Mouse monoclonal clone 2B1 anti-SMN antibody recognizes human, mouse, and Xenopus survival of motor neurons proteins.

特異性

The antibody recognizes human, mouse, and Xenopus SMN.

免疫原

recombinant human SMN

アプリケーション

Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Enzyme-linked immunosorbent assay (1 paper)
Mouse monoclonal clone 2B1 anti-SMN antibody is an important tool for studying the role of the survival of motor neurons protein in nuclear processes and spinal muscular atrophy (SMA). It may be used in immunoblotting (~35 kDa), immunoprecipitation, and immunocytochemistry.

生物化学的/生理学的作用

Survival of Motor Neurons (SMN) complex is important in various biological processes, such as assembly and restructuring of spliceosomal small nuclear ribonucleoproteins (snRNPs), pre mRNA splicing and transcription. Spinal muscular atrophy (SMA) is caused by reduced expression or mutations in the survival of motor neurons (SMN) protein. Deletion or mutation in the telomeric copy (SMN1) causes the SMA phenotype. The severity of SMA is in direct correlation with the expression level of the SMN protein, either from the SMN1 gene or a different spliced form of SMN from the SMN2 gene. The SMN complex interacts with various protein substrates such as Sm and Lsm proteins of the spliceosomal snRNPs, fibrillarin, GAR1, RNA helicase A, the human hnRNP proteins (hnRNPQ, U and R), coilin and p53.

物理的形状

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15mM sodium azide.

保管および安定性

For continuous use, store at 2-8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in "frostfree" freezers, is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.

免責事項

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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保管分類コード

12 - Non Combustible Liquids

WGK

WGK 2

引火点(°F)

Not applicable

引火点(℃)

Not applicable


適用法令

試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。

Jan Code

S2944-BULK:
S2944-200UL:
S2944-VAR:


試験成績書(COA)

製品のロット番号・バッチ番号を入力して、試験成績書(COA) を検索できます。ロット番号・バッチ番号は、製品ラベルに「Lot」または「Batch」に続いて記載されています。

以前この製品を購入いただいたことがある場合

文書ライブラリで、最近購入した製品の文書を検索できます。

文書ライブラリにアクセスする

Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?
Burghes AHM and Beattie CE
Nature Reviews. Neuroscience, 10(8), 597-597 (2009)
Dione T Kobayashi et al.
PloS one, 6(8), e24269-e24269 (2011-09-10)
Genetic defects leading to the reduction of the survival motor neuron protein (SMN) are a causal factor for Spinal Muscular Atrophy (SMA). While there are a number of therapies under evaluation as potential treatments for SMA, there is a critical
Q Liu et al.
The EMBO journal, 15(14), 3555-3565 (1996-07-15)
Spinal muscular atrophy (SMA) is a common, often fatal, autosomal recessive disease leading to progressive muscle wasting and paralysis as a result of degeneration of anterior horn cells of the spinal cord. A gene termed survival of motor neurons (SMN)
Thomas O Crawford et al.
PloS one, 7(4), e33572-e33572 (2012-05-05)
The universal presence of a gene (SMN2) nearly identical to the mutated SMN1 gene responsible for Spinal Muscular Atrophy (SMA) has proved an enticing incentive to therapeutics development. Early disappointments from putative SMN-enhancing agent clinical trials have increased interest in
Sergey Paushkin et al.
Current opinion in cell biology, 14(3), 305-312 (2002-06-18)
Spinal muscular atrophy is a common, often lethal, neurodegenerative disease that results from low levels of, or loss-of-function mutations in, the SMN (survival of motor neurons) protein. SMN oligomerizes and forms a stable complex with five additional proteins: Gemins 2-6.

ライフサイエンス、有機合成、材料科学、クロマトグラフィー、分析など、あらゆる分野の研究に経験のあるメンバーがおります。.

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