HPA000966

抗GLA ウサギ宿主抗体

Ab2, Prestige Antibodies^® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

• 別名: 抗α-D-ガラクトシダーゼA抗体 ウサギ宿主抗体, 抗α-D-ガラクトシドガラクトヒドロラーゼ抗体 ウサギ宿主抗体, 抗α-ガラクトシダーゼA前駆体抗体 ウサギ宿主抗体, 抗アガルシダーゼα抗体 ウサギ宿主抗体, 抗メリビアーゼ抗体 ウサギ宿主抗体
• UNSPSCコード: 12352203
• Human Protein Atlas Number: HPA000966
• NACRES: NA.41

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
• 交差性: human
• 強化検証: orthogonal RNAseq; independent; Learn more about Antibody Enhanced Validation
• テクニック: immunohistochemistry: 1:200- 1:500
• 免疫原配列: FDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMA
• UniProtアクセッション番号: P06280
• 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 ... GLA(2717)

詳細

詳細

GLA (galactosidase α) gene is localized to human chromosome Xq22.1, which codes for a precursor protein of 429 amino acids. This protein is then processed and glycosylated to form a mature protein of 398 amino acids. This protein is localized to lysosomes and exists as a homodimer.

免疫原

α-ガラクトシダ-ゼA前駆体のPrEST (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。

生物化学的／生理学的作用

α-galactosidase is a glycoside hydrolase enzyme that hydrolyses the terminal α-galactosyl moieties from glycolipids and glycoproteins, encoded by the GLA gene. Deficit of α-galactosidase A enzyme causes Fabry disease, an X-linked inherited metabolic condition (leads to glycosphingolipid storage, mainly globotriaosylceramide). Missense mutations in the α-galactosidase A (GLA) gene comprises the majority of mutations that are responsible for Fabry disease.

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 APREST74068

物理的形状

PBS溶液 (pH 7.2, 40%グリセロ-ルおよび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)