Anti-Galactosidase alpha 抗体 (ab169315)
Key features and details
- Mouse polyclonal to Galactosidase alpha
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
リコンビナント抗体で、ロット間での高い再現性を実現
- 異なるロット間での安定した再現性
- 容易なスケールアップ
- 評価試験による特異性の確認済み
- 倫理基準に準拠 - アニマル・フリーの生産
製品の概要
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製品名
Anti-Galactosidase alpha antibody
Galactosidase alpha 一次抗体 製品一覧 -
製品の詳細
Mouse polyclonal to Galactosidase alpha -
由来種
Mouse -
アプリケーション
適用あり: WBmore details -
種交差性
交差種: Human -
免疫原
Recombinant full length protein within Human Galactosidase alpha aa 1-450. The exact immunogen sequence used to generate this antibody is proprietary information. If additional detail on the immunogen is needed to determine the suitability of the antibody for your needs, please contact our Scientific Support team to discuss your requirements.
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ポジティブ・コントロール
- Galactosidase alpha transfected 293T cell lysate.
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特記事項
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. -
バッファー
pH: 7.4
Constituent: 100% PBS -
Concentration information loading...
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精製度
Protein A purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
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Related Products
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab169315の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
アプリケーション | Abreviews | 特記事項 |
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WB |
Use a concentration of 1 µg/ml. Predicted molecular weight: 49 kDa.
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特記事項 |
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WB
Use a concentration of 1 µg/ml. Predicted molecular weight: 49 kDa. |
ターゲット情報
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関連疾患
Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. -
配列類似性
Belongs to the glycosyl hydrolase 27 family. -
細胞内局在
Lysosome. - Information by UniProt
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参照データベース
- Entrez Gene: 2717 Human
- Omim: 300644 Human
- SwissProt: P06280 Human
- Unigene: 69089 Human
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別名
- AGAL_HUMAN antibody
- Agalsidase alfa antibody
- Alpha D galactosidase A antibody
see all
画像
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All lanes : Anti-Galactosidase alpha antibody (ab169315) at 1 µg/ml
Lane 1 : Galactosidase alpha transfected 293T cell lysate
Lane 2 : Non-transfected 293T cell lysate
Lysates/proteins at 15 µl per lane.
Secondary
All lanes : Goat Anti-Mouse IgG (H&L)-HRP at 1/2500 dilution
Developed using the ECL technique.
Predicted band size: 49 kDa
データシートおよび資料
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Datasheet download
参考文献 (1)
ab169315 は 1 報の論文で使用されています。
- Bohl C et al. Fluorescent probes for selective protein labeling in lysosomes: a case of a-galactosidase A. FASEB J 31:5258-5267 (2017). PubMed: 28821638