Anti-GAA 抗体 (ab102815)
Key features and details
- Rabbit polyclonal to GAA
- Suitable for: WB
- Reacts with: Mouse, 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-GAA antibody
GAA 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to GAA -
由来種
Rabbit -
アプリケーション
適用あり: WBmore details -
種交差性
交差種: Mouse, Human -
免疫原
Recombinant full length protein within Human GAA aa 1-1000. 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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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. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
バッファー
pH: 7.4
Constituent: 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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Assay kits
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Compatible Secondaries
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Isotype control
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Recombinant Protein
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ab102815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 | (1) |
1/500 - 1/1000. Predicted molecular weight: 105 kDa.
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特記事項 |
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WB
1/500 - 1/1000. Predicted molecular weight: 105 kDa. |
ターゲット情報
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機能
Essential for the degradation of glygogen to glucose in lysosomes. -
関連疾患
Defects in GAA are the cause of glycogen storage disease type 2 (GSD2) [MIM:232300]; also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy. -
配列類似性
Belongs to the glycosyl hydrolase 31 family.
Contains 1 P-type (trefoil) domain. -
翻訳後修飾
The different forms of acid glucosidase are obtained by proteolytic processing.
Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor. -
細胞内局在
Lysosome. Lysosome membrane. - Information by UniProt
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参照データベース
- Entrez Gene: 2548 Human
- Entrez Gene: 14387 Mouse
- Omim: 606800 Human
- SwissProt: P10253 Human
- SwissProt: P70699 Mouse
- Unigene: 1437 Human
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別名
- 70 kDa lysosomal alpha-glucosidase antibody
- Acid alpha glucosidase antibody
- Acid maltase antibody
see all
画像
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Anti-GAA antibody (ab102815) at 1/500 dilution + Mouse intestine lysate at 50 µg
Predicted band size: 105 kDa -
All lanes : Anti-GAA antibody (ab102815) at 1/500 dilution
Lane 1 : GAA transfected 293T cell line
Lane 2 : Non transfected 293T cell line
Lysates/proteins at 25 µg per lane.
Predicted band size: 105 kDa
データシートおよび資料
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Datasheet download
参考文献 (0)
ab102815 は論文での使用が確認できていません。