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  1. Link

    gaa-antibody-ab102815.pdf

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Signal Transduction Metabolism Energy Metabolism
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Anti-GAA 抗体 (ab102815)

  • Datasheet
Reviews (1)Q&A (4)

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Western blot - Anti-GAA antibody (ab102815)
  • Western blot - Anti-GAA antibody (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

Product image
Anti-GAA antibody [EPR4716(2)] (ab137068)
  • 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

  • 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.

  • 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

  • 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...
  • 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

    • Signal Transduction
    • Metabolism
    • Energy Metabolism
    • Metabolism
    • Pathways and Processes
    • Metabolic signaling pathways
    • Energy transfer pathways
    • Energy Metabolism
    • Metabolism
    • Types of disease
    • Cancer
    • Neuroscience
    • Processes

関連製品

  • Assay kits

    • Alpha-Glucosidase Activity Assay Kit (Colorimetric) (ab174093)
  • Compatible Secondaries

    • Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (ab150077)
    • Goat Anti-Rabbit IgG H&L (HRP) (ab205718)
  • Isotype control

    • Rabbit IgG, polyclonal - Isotype Control (ChIP Grade) (ab171870)
  • Recombinant Protein

    • Recombinant Human GAA protein (ab114893)

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
WB (1)
1/500 - 1/1000. Predicted molecular weight: 105 kDa.
特記事項
WB
1/500 - 1/1000. Predicted molecular weight: 105 kDa.

ターゲット情報

  • 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.
  • Target information above from: UniProt accession P10253 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • 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
    • 別名

      • 70 kDa lysosomal alpha-glucosidase antibody
      • Acid alpha glucosidase antibody
      • Acid maltase antibody
      • Aglucosidase alfa antibody
      • Alpha glucosidase antibody
      • GAA antibody
      • Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody
      • Glucosidase alpha acid antibody
      • Glucosidase alpha antibody
      • LYAG antibody
      • LYAG_HUMAN antibody
      • Lysosomal alpha glucosidase antibody
      see all

    画像

    • Western blot - Anti-GAA antibody (ab102815)
      Western blot - Anti-GAA antibody (ab102815)
      Anti-GAA antibody (ab102815) at 1/500 dilution + Mouse intestine lysate at 50 µg

      Predicted band size: 105 kDa

    • Western blot - Anti-GAA antibody (ab102815)
      Western blot - Anti-GAA antibody (ab102815)
      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

    プロトコール

    • Western blot protocols

    Click here to view the general protocols

    データシートおよび資料

    • Datasheet download

      Download

    参考文献 (0)

    ab102815 を使用した論文を発表された方は、こちらまでお知らせください。データシートに掲載させていただきます。

    ab102815 は論文での使用が確認できていません。

    レビューと Q&A

    Show All レビュー Q&A
    レビューを送る 質問を送る

    1-5 of 5 Abreviews or Q&A

    Western blot abreview for Anti-GAA antibody

    Excellent
    Abreviews
    Abreviews
    Application
    Western blot
    Sample
    Human Purified protein (N/A)
    Gel Running Conditions
    Reduced Denaturing (4-12%)
    Loading amount
    50 µg
    Treatment
    N/A
    Specification
    N/A
    Blocking step
    Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 3% · Temperature: 25°C
    Read More
    The reviewer received a reward from Abcam’s Loyalty Program in thanks for submitting this Abreview and for helping the scientific community make better-informed decisions.

    Abcam user community

    Verified customer

    投稿 Dec 30 2019

    Question

    Thank you for contacting me.

    I have used different buffers to block the membrane and to incubate the primary antibody O/N respect to your suggestions.

    So I would to try to run a gel and to perform a WB in your condition.

    I’ll let you know soon

    Thank you very much

    Best regards

    Read More

    Abcam community

    Verified customer

    Asked on Apr 20 2012

    Answer

    That seems like a good idea.

    I would possible also suggest reducing the washing conditions used slightly, using 3x5 minute washes with TBST rather than extended periods.

    Please do let me know how you get on.

    Read More

    Abcam Scientific Support

    Answered on Apr 20 2012

    Question

    Inquiry: I have used the GAA antibody on mouse liver tissue , left ventricle tissue , right ventricle and mouse intestine tissue. Protein loaded is 50 micrograms Blocking condition is TBS-T tween 0.1% + milk 5% ECL PLUS SDS PAGE AND WET TRANSFER 1:500 GAA diluition in TBS-T +5% BSA O/n at 4°C with agitation secondary anti rabbit 1:5000 1h at Tr 3 Step of wash (10') after primary antibody and after secondary antibody . I would help and advice because I exclude a problem with ECL Secondary antibody or transfer. I have used an other antibody for GAPDH protein that works very well. What is the buffer that you suggest for the incubation with GAA primary antibody?

    Read More

    Abcam community

    Verified customer

    Asked on Apr 20 2012

    Answer

    Thank you for contacting us.

    I now have some further information in regards to how the western blot presented on the datasheet of Anti-GAA antibody (ab102815) was performed.

    5% non-fat milk in PBST (0.2%) was used to block the membrane for 1 hour a room temperature.

    The working concentration of the primary antibody was 1ug/mL and theprimary antibody was diluted in fresh blocking buffer [5% non-fat milk/PBST (0.2%)]. The antibody wasincubatedovernight at4℃.

    It therefore seems that the blocking and diluent you have been using should not be contributing to the problem. If you could pleaseprovide the information requested inmy previous email hopefully I can try to isolate where the problem may be coming from, and if a replacement antibody may be required.

    From my previous email:

    I was hoping you may be able to clarify a little further the problems you have been experiencing. Do you observe no bands at all or is there high background? Would it be possible to send an image of the results obtained? I also have a few further questions:

    1. how was the tissue samples prepared? How were they lysed and were any protease inhibitors used?

    2. Was the sample run denatured or native? Was boiling and reducing agents employed?

    3. What buffer was employed in the wash steps?

    4. Do you have the order number (or purchase order number) of ab102815?

    This information will also allow us to investigate this case internally and initiate additional testing where necessary. If the product was purchased in the last six months and is being used according to our Abpromise, we would be happy to replace or refund the antibody.

    I look forward to receiving your reply.

    Read More

    Abcam Scientific Support

    Answered on Apr 20 2012

    Question

    Inquiry: I have used the GAA antibody on mouse liver tissue , left ventricle tissue , right ventricle and mouse intestine tissue. Protein loaded is 50 micrograms Blocking condition is TBS-T tween 0.1% + milk 5% ECL PLUS SDS PAGE AND WET TRANSFER 1:500 GAA diluition in TBS-T +5% BSA O/n at 4°C with agitation secondary anti rabbit 1:5000 1h at Tr 3 Step of wash (10') after primary antibody and after secondary antibody . I would help and advice because I exclude a problem with ECL Secondary antibody or transfer. I have used an other antibody for GAPDH protein that works very well. What is the buffer that you suggest for the incubation with GAA primary antibody?

    Read More

    Abcam community

    Verified customer

    Asked on Apr 19 2012

    Answer

    Thank you for contacting us.

    I am sorry to hear you are experiencing difficulties with one of our products. We take product complaints very seriously, and investigate every product that we feel may not be performing correctly.

    I was hoping you may be able to clarify a little further the problems you have been experiencing. Do you observe no bands at all or is there high background? Would it be possible to send an image of the results obtained? I also have a few further questions:

    1. how was the tissue samples prepared? How were they lysed and were any protease inhibitors used?

    2. Was the sample run denatured or native? Was boiling and reducing agents employed?

    3. Whatbuffer was employed in the wash steps?

    4. Do you have the order number (or purchase order number) of ab102815?

    This information will also allow us to investigate this case internally and initiate additional testing where necessary. If the product was purchased in the last six months and is being used according to our Abpromise, we would be happy to replace or refund the antibody.

    I look forward to receiving your reply.

    Read More

    Abcam Scientific Support

    Answered on Apr 19 2012

    Question

    I have used the GAA antibody on mouse liver tissue , left ventricle tissue , right ventricle and mouse intestine tissue.

    Protein loaded is 50 micrograms

    Blocking condition is TBS-T tween 0.1% + milk 5%

    ECL PLUS

    SDS PAGE AND WET TRANSFER

    1:500 GAA diluition in TBS-T +5% BSA O/n at 4°C with agitation

    secondary anti rabbit 1:5000 1h at Tr

    3 Step of wash (10') after primary antibody and after secondary antibody .

    I would help and advice because I exclude a problem with ECL , secondary antibody or transfer.

    I have used an other antibody for GAPDH protein that works very well.

    What is the buffer that you suggest for the incubation with GAA primary antibody?



    GAA antibody is ab102815

    Read More

    Abcam community

    Verified customer

    Asked on Apr 19 2012

    Answer

    Thank you for contacting us.

    I am sorry to hear you are experiencing difficulties with one of our products. We take product complaints very seriously, and investigate every product that we feel may not be performing correctly.

    I am attaching our questionnaire so that we can gather further information regarding the samples tested and the protocol used. Once we receive the completed questionnaire, we will look at the protocol and see if there are any suggestions we can make that may improve the results. This information will also allow us to investigate this case internally and initiate additional testing where necessary. If the product was purchased in the last six months and is being used according to our Abpromise, we would be happy to replace or refund the antibody.


    I look forward to receiving your reply.

    Read More

    Abcam Scientific Support

    Answered on Apr 19 2012

    Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
    For licensing inquiries, please contact partnerships@abcam.com

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