HPA010008

抗MTM1抗体 ウサギ宿主抗体

Ab1, Prestige Antibodies^® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

• 別名: 抗ミオチュブラリン抗体 ウサギ宿主抗体
• UNSPSCコード: 12352203
• Human Protein Atlas Number: HPA010008
• NACRES: NA.43
• 結合体: unconjugated
• application: IF; IHC
• クローン: polyclonal
• 化学種の反応性: human
• citations: 5
• テクニック: immunofluorescence: 0.25-2 μg/mL; immunohistochemistry: 1:500-1:1000

特性

• 由来生物: rabbit
• 品質水準: 100
• 結合体: unconjugated
• 抗体製品の状態: affinity isolated antibody
• 抗体製品タイプ: primary antibodies
• クローン: polyclonal
• 製品種目: Prestige Antibodies^® Powered by Atlas Antibodies
• フォーム: buffered aqueous glycerol solution
• 化学種の反応性: human
• テクニック: immunofluorescence: 0.25-2 μg/mL; immunohistochemistry: 1:500-1:1000
• 免疫原配列: KFNVDGWTVYNPVEEYRRQGLPNHHWRITFINKCYELCDTYPALLVVPYRASDDDLRRVATFRSRNRIPVLSWIHPENKTVIVRCSQPLVGMSGKRNKDDEKYLDVIRETNKQISKLTIYDARPSVNAVANK
• UniProtアクセッション番号: Q13496
• 輸送温度: wet ice
• 保管温度: −20°C
• ターゲットの翻訳後修飾: unmodified
• 遺伝子情報: human ... MTM1(4534)

詳細

詳細

MTM1 (myotubularin 1) is a lipid phosphatase, which is abundant at the junctional SR (sarcoplasmic reticulum) of the muscle triads. It is a member of conserved phosphatase family myotubularin, which contains both active phosphatases and inactive myotubularin-related proteins (MTMRs).

免疫原

ミオチュブラリンのPrEST(recombinant protein epitope signature tag)抗原リコンビナントタンパク質。

アプリケーション

Prestige抗体^®は、Human Proteome Resource(HPR)プロジェクト(www.proteinatlas.org)によって開発・実証されたAtlas抗体です。抗体はすべて、数百の正常組織・疾病組織に対する免疫組織染色試験を行っています。これらの染色画像はHuman Protein Atlas(HPA)サイトで[Image Gallery]リンクをクリックするとご覧いただけます。さらに、ほとんどのPrestige抗体はプロテインアレイおよびウェスタンブロッティングの試験を行っています。試験のプロトコールおよびPrestige抗体、HPAに関する情報はsigma.com/prestigeをご覧ください。

Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Immunofluorescence (1 paper)

生物化学的／生理学的作用

MTM1 (myotubularin 1) protein plays an essential role in facilitating the curvature of SR (sarcoplasmic reticulum) membrane and SR remodelling. Therefore, it is critical for homeostasis of calcium and excitation-contraction coupling. The major function of this protein is the dephosphorylation of phosphatidylinositol-3-phosphate (PtdIns3P) and phosphatidylinositol-3,5-bisphosphate (PtdIns(3,5P)₂). Mutations in this gene are linked to X-linked myotubular myopathy (XLMTM), which is an inherited disorder characterized by severe generalized hypotonia and skeletal muscles weakness with respiratory insufficiency. Mostly this disease is fatal in the initial months of life. Heterozygous mutations resulting in abnormal splicing in this gene are associated with early onset, slowly progressive myopathy. c.431delT (p.Leu144fs) mutation in this gene results in bilateral subdural hygromas and cephalhaematomas in male twins suffering from severe myotubular myopathy.

特徴および利点

Prestige Antibodies^® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies^® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:

• IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
• Protein array of 364 human recombinant protein fragments.

関連事項

Corresponding Antigen APREST74421

物理的形状

PBS溶液 (pH 7.2, 40%グリセロ-ル, 0.02%アジ化ナトリウム含有)

法的情報

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

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.

安全性情報

• 保管分類コード: 10 - Combustible liquids
• WGK: WGK 1
• 引火点(°F): Not applicable
• 引火点(℃): Not applicable
• 個人用保護具 (PPE): Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)