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由来生物
mouse
品質水準
抗体製品の状態
purified immunoglobulin
抗体製品タイプ
primary antibodies
クローン
2B1, monoclonal
化学種の反応性
human
化学種の反応性（ホモロジーによる予測）
Xenopus, mouse
テクニック
immunocytochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable
アイソタイプ
IgG1κ
NCBIアクセッション番号
UniProtアクセッション番号
輸送温度
wet ice
ターゲットの翻訳後修飾
unmodified
遺伝子情報
human ... SMA4(11039)
詳細
The survival of motor neurons (SMN) protein is essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery. Though it is ubiquitously expressed, SMN deficiency causes the motor neuron degenerative disease spinal muscular atrophy (SMA). SMN deficiency has unexpected cell type-specific effects on the repertoire of snRNAs and mRNAs. It alters the stoichiometry of snRNAs and causes widespread pre-mRNA splicing defects in numerous transcripts of diverse genes, preferentially those containing a large number of introns, in SMN-deficient mouse tissues. The SMN complex plays a role in RNA metabolism and in splicing regulation.
特異性
This antibody recognizes SMN.
免疫原
Epitope: Unknown
His-tagged recombinant protein corresponding to human SMN.
アプリケーション
Research Category
エピジェネティクス及び核内機能分子
エピジェネティクス及び核内機能分子
Research Sub Category
RNA代謝及び結合タンパク質
RNA代謝及び結合タンパク質
RNA代謝及び結合タンパク質
RNA代謝及び結合タンパク質
Anti-SMN Antibody, clone 2B1 is a Mouse Monoclonal Antibody for detection of SMN also known as Spinal muscular atrophy (Werdnig-Hoffmann disease Kugelberg-Welander disease) & has been validated in WB, ICC & IP.
Immunocytochemistry Analysis: 1:500 dilution from a representative lot detected SMN in HeLa cells.
品質
Evaluated by Western Blot in HeLa cell lysate.
Western Blot Analysis: 0.5 µg/ml of this antibody detected SMN on 10 µg of HeLa cell lysate.
Western Blot Analysis: 0.5 µg/ml of this antibody detected SMN on 10 µg of HeLa cell lysate.
ターゲットの説明
35 kDa was observed; however, the calculated molecular weight is 31.849 kDa
物理的形状
Protein G Purified
Format: Purified
Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide.
保管および安定性
Stable for 1 year at 2-8°C from date of receipt.
アナリシスノート
Control
HeLa cell lysate
HeLa cell lysate
その他情報
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
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 1
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
05-1532:
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。
Minoru Satoh et al.
Arthritis and rheumatism, 63(7), 1972-1978 (2011-03-23)
Autoantibodies in the systemic rheumatic diseases are clinically useful biomarkers of the diagnosis or of certain clinical characteristics. An unusual pattern of immunoprecipitation, in which the D, E, F, and G proteins of small nuclear RNPs (snRNP) but without other
Lea Marie Esser et al.
International journal of molecular sciences, 24(21) (2023-11-14)
The survival motor neuron (SMN) complex is a multi-megadalton complex involved in post-transcriptional gene expression in eukaryotes via promotion of the biogenesis of uridine-rich small nuclear ribonucleoproteins (UsnRNPs). The functional center of the complex is formed from the SMN/Gemin2 subunit.
Elena Hernandez-Gerez et al.
Annals of clinical and translational neurology, 7(9), 1580-1593 (2020-08-14)
The purpose of the study was to determine the extent and role of systemic hypoxia in the pathogenesis of spinal muscular atrophy (SMA). Hypoxia was assayed in vivo in early-symptomatic (postnatal day 5) SMA-model mice by pimonidazole and [18 F]-Fluoroazomycin
Lu Chen et al.
Nucleic acids research, 50(21), 12400-12424 (2022-08-11)
Trimethylguanosine synthase 1 (TGS1) is a highly conserved enzyme that converts the 5'-monomethylguanosine cap of small nuclear RNAs (snRNAs) to a trimethylguanosine cap. Here, we show that loss of TGS1 in Caenorhabditis elegans, Drosophila melanogaster and Danio rerio results in
Phosphorylation of pICln by the autophagy activating kinase ULK1 regulates snRNP biogenesis and splice activity of the cell.
Esser, et al.
Computational and structural biotechnology journal, 21, 2100-2109 (2023)
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