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遺伝子ノックアウト細胞株 検証済

Anti-GBA 抗体 [2E2] (ab55080)

  • Datasheet
Reviews (3)Q&A (7)References (14)

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Abpromise

保証された製品品質、優れたカスタマー・サポート。 詳細はこちら。

Western blot - Anti-GBA antibody [2E2] (ab55080)
  • Western blot - Anti-GBA antibody [2E2] (ab55080)
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GBA antibody [2E2] (ab55080)
  • Western blot - Anti-GBA antibody [2E2] (ab55080)

Key features and details

  • Mouse monoclonal [2E2] to GBA
  • Suitable for: WB, IHC-P
  • Knockout validated
  • Reacts with: Human
  • Isotype: IgG2a

こちらの製品もご検討ください

タンパク質
Product image
Recombinant Mouse GBA protein (Tagged) (ab235724)
ノックアウト
Product image
Human GBA knockout HeLa cell line (ab265038)
アッセイ
Product image
Glucosylceramidase Activity Assay Kit (Fluorometric) (ab273339)

関連製品

製品の概要

  • 製品名

    Anti-GBA antibody [2E2]
    GBA 一次抗体 製品一覧
  • 製品の詳細

    Mouse monoclonal [2E2] to GBA
  • 由来種

    Mouse
  • アプリケーション

    適用あり: WB, IHC-Pmore details
  • 種交差性

    交差種: Human
  • 免疫原

    Recombinant fragment (GST-tag) corresponding to Human GBA aa 146-236.
    Sequence:

    SYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKL KIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGS

    Run BLAST with BLAST the sequence with ExPASy Run BLAST with BLAST the sequence with NCBI
  • 特記事項

    This product was changed from ascites to tissue culture supernatant on 15 May 2019. Please note that the dilutions may need to be adjusted accordingly. If you have any questions, please do not hesitate to contact our scientific support team.

    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 and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • バッファー

    pH: 7.4
  • Concentration information loading...
  • 精製度

    Protein A purified
  • ポリ/モノ

    モノクローナル
  • クローン名

    2E2
  • アイソタイプ

    IgG2a
  • 軽鎖の種類

    kappa
  • 研究分野

    • Neuroscience
    • Neurology process
    • Neurodegenerative disease
    • Parkinson's disease
    • Other
    • Signal Transduction
    • Metabolism
    • Energy Metabolism
    • Signal Transduction
    • Metabolism
    • Lipid metabolism
    • Cancer
    • Cancer Metabolism
    • Metabolic signaling pathway
    • Metabolism of lipids and lipoproteins
    • Metabolism
    • Pathways and Processes
    • Metabolic signaling pathways
    • Lipid and lipoprotein metabolism
    • Lipid metabolism
    • Metabolism
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    • Metabolic signaling pathways
    • Energy transfer pathways
    • Energy Metabolism
    • Metabolism
    • Types of disease
    • Neurodegenerative disease
    • Metabolism
    • Types of disease
    • Cancer
    • Neuroscience
    • Processes

関連製品

  • Compatible Secondaries

    • Goat Anti-Mouse IgG H&L (Alexa Fluor® 488) (ab150113)
    • Goat Anti-Mouse IgG H&L (HRP) (ab205719)
  • Isotype control

    • Mouse IgG2a, kappa monoclonal [MG2a-53] - Isotype control (ab18415)
  • Recombinant Protein

    • Recombinant Mouse GBA protein (Tagged) (ab235724)

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ab55080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 (2)
Use at an assay dependent concentration. Predicted molecular weight: 60 kDa.
IHC-P
Use at an assay dependent concentration.
特記事項
WB
Use at an assay dependent concentration. Predicted molecular weight: 60 kDa.
IHC-P
Use at an assay dependent concentration.

ターゲット情報

  • 関連疾患

    Defects in GBA are the cause of Gaucher disease (GD) [MIM:230800]; also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Different clinical forms are recognized depending on the presence (neuronopathic forms) or absence of central nervous system involvement, severity and age of onset.
    Defects in GBA are the cause of Gaucher disease type 1 (GD1) [MIM:230800]; also known as adult non-neuronopathic Gaucher disease. GD1 is characterized by hepatosplenomegaly with consequent anemia and thrombopenia, and bone involvement. The central nervous system is not involved.
    Defects in GBA are the cause of Gaucher disease type 2 (GD2) [MIM:230900]; also known as acute neuronopathic Gaucher disease. GD2 is the most severe form and is universally progressive and fatal. It manifests soon after birth, with death generally occurring before patients reach two years of age.
    Defects in GBA are the cause of Gaucher disease type 3 (GD3) [MIM:231000]; also known as subacute neuronopathic Gaucher disease. GD3 has central nervous manifestations.
    Defects in GBA are the cause of Gaucher disease type 3C (GD3C) [MIM:231005]; also known as pseudo-Gaucher disease or Gaucher-like disease.
    Defects in GBA are the cause of Gaucher disease perinatal lethal (GDPL) [MIM:608013]. It is a distinct form of Gaucher disease type 2, characterized by fetal onset. Hydrops fetalis, in utero fetal death and neonatal distress are prominent features. When hydrops is absent, neurologic involvement begins in the first week and leads to death within 3 months. Hepatosplenomegaly is a major sign, and is associated with ichthyosis, arthrogryposis, and facial dysmorphism.
    Note=Perinatal lethal Gaucher disease is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
    Defects in GBA contribute to susceptibility to Parkinson disease (PARK) [MIM:168600]. A complex neurodegenerative disorder characterized by bradykinesia, resting tremor, muscular rigidity and postural instability. Additional features are characteristic postural abnormalities, dysautonomia, dystonic cramps, and dementia. The pathology of Parkinson disease involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain. The disease is progressive and usually manifests after the age of 50 years, although early-onset cases (before 50 years) are known. The majority of the cases are sporadic suggesting a multifactorial etiology based on environmental and genetic factors. However, some patients present with a positive family history for the disease. Familial forms of the disease usually begin at earlier ages and are associated with atypical clinical features.
  • 配列類似性

    Belongs to the glycosyl hydrolase 30 family.
  • 細胞内局在

    Lysosome membrane. Interaction with saposin-C promotes membrane association.
  • Target information above from: UniProt accession P04062 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: 2629 Human
    • Omim: 606463 Human
    • SwissProt: P04062 Human
    • Unigene: 282997 Human
    • Unigene: 719930 Human
    • 別名

      • Acid beta glucosidase antibody
      • Acid beta-glucosidase antibody
      • Alglucerase antibody
      • Beta glucocerebrosidase antibody
      • BETA GLUCOSIDASE, ACID antibody
      • Beta-glucocerebrosidase antibody
      • betaGC antibody
      • D glucosyl N acylsphingosine glucohydrolase antibody
      • D-glucosyl-N-acylsphingosine glucohydrolase antibody
      • EC 3.2.1.45 antibody
      • GBA antibody
      • Gba protein antibody
      • GBA1 antibody
      • GC antibody
      • GCase antibody
      • GCB antibody
      • GLCM_HUMAN antibody
      • GLUC antibody
      • Glucocerebrosidase (alt.) antibody
      • Glucocerebrosidase antibody
      • GLUCOCEREBROSIDASE PSEUDOGENE antibody
      • Glucosidase beta antibody
      • Glucosidase, beta, acid antibody
      • Glucosidase, beta; acid (includes glucosylceramidase) antibody
      • Glucosylceramidase antibody
      • Imiglucerase antibody
      • Lysosomal glucocerebrosidase antibody
      • OTTHUMP00000033992 antibody
      • OTTHUMP00000033993 antibody
      see all

    画像

    • Western blot - Anti-GBA antibody [2E2] (ab55080)
      Western blot - Anti-GBA antibody [2E2] (ab55080)

      Lane 1: Wild-type HAP1 whole cell lysate (40 µg)
      Lane 2: GBA knockout HAP1 whole cell lysate (40 µg)
      Lane 3: MCF7 whole cell lysate (40 µg)
      Lane 4: HepG2 whole cell lysate (40 µg) 

      Lanes 1 - 4: Merged signal (red and green). Green - ab55080 observed at 70 kDa. Red - loading control, ab181602, observed at 37 kDa. 

      ab55080 was shown to specifically react with GBA in wild-type HAP1 cells along with additional cross-reactive bands. No bands were observed when GBA knockout samples were used. Wild-type and GBA knockout samples were subjected to SDS-PAGE. Ab55080 and ab181602 (Rabbit anti GAPDH loading control) were incubated overnight at 4°C at 1 ug/ml and 1/10,000 dilution respectively. Blots were developed with Goat anti-Mouse IgG H&L (IRDye® 800CW) preabsorbed (ab216772) and Goat anti-Rabbit IgG H&L (IRDye® 680RD) preabsorbed (ab216777) secondary antibodies at 1/10,000 dilution for 1 hour at room temperature before imaging.

      This image was generated using the ascites version of the product.

    • Western blot - Anti-GBA antibody [2E2] (ab55080)
      Western blot - Anti-GBA antibody [2E2] (ab55080)

      GBA antibody (ab55080) at 1ug/lane + MCF-7 cell lysate at 25ug/lane.

      This image was generated using the ascites version of the product.

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GBA antibody [2E2] (ab55080)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GBA antibody [2E2] (ab55080)

      GBA antibody (ab55080) used in immunohistochemistry at 3ug/ml on formalin fixed and paraffin embedded human breast cancer.

      This image was generated using the ascites version of the product.

    • Western blot - Anti-GBA antibody [2E2] (ab55080)
      Western blot - Anti-GBA antibody [2E2] (ab55080)This image is a courtesy of Anonymous Abreview
      All lanes : Anti-GBA antibody [2E2] (ab55080) at 1/1000 dilution

      Lane 1 : Lysate prepared from MOCK
      Lane 2 : Lysate prepared from human HN10 cells

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : IRDye® donkey polyclonal to mouse IgG at 1/3000 dilution

      Performed under reducing conditions.

      Predicted band size: 60 kDa
      Observed band size: 60 kDa


      Exposure time: 1 minute


      This image was generated using the ascites version of the product.

      See Abreview

    プロトコール

    • Immunohistochemistry protocols
    • Immunocytochemistry & immunofluorescence protocols
    • 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 (14)

    ab55080 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。

    ab55080 は 14 報の論文で使用されています。

    • Galvagnion C  et al. Sphingolipid changes in Parkinson L444P GBA mutation fibroblasts promote α-synuclein aggregation. Brain 145:1038-1051 (2022). PubMed: 35362022
    • Glajch KE  et al. Wild-type GBA1 increases the a-synuclein tetramer-monomer ratio, reduces lipid-rich aggregates, and attenuates motor and cognitive deficits in mice. Proc Natl Acad Sci U S A 118:N/A (2021). PubMed: 34326260
    • Sucunza D  et al. Glucocerebrosidase Gene Therapy Induces Alpha-Synuclein Clearance and Neuroprotection of Midbrain Dopaminergic Neurons in Mice and Macaques. Int J Mol Sci 22:N/A (2021). PubMed: 34062940
    • Sanyal A  et al. LRRK2 Kinase Inhibition Rescues Deficits in Lysosome Function Due to Heterozygous GBA1 Expression in Human iPSC-Derived Neurons. Front Neurosci 14:442 (2020). PubMed: 32499675
    • Sanyal A  et al. Lysosome and Inflammatory Defects in GBA1-Mutant Astrocytes Are Normalized by LRRK2 Inhibition. Mov Disord 35:760-773 (2020). PubMed: 32034799
    View all Publications for this product

    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

    1-10 of 10 Abreviews or Q&A

    Western blot abreview for Anti-GBA antibody

    Good
    Abreviews
    Abreviews
    abreview image
    Application
    Western blot
    Sample
    Rat Tissue lysate - whole (Brain)
    Loading amount
    20 µg
    Specification
    Brain
    Gel Running Conditions
    Reduced Denaturing (10% SDS-PAGE)
    Blocking step
    Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 24°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.

    DR. Ruma Raha-Chowdhury

    Verified customer

    投稿 Sep 07 2010

    Immunohistochemistry (Frozen sections) abreview for Anti-GBA antibody

    Excellent
    Abreviews
    Abreviews
    abreview image
    Application
    Immunohistochemistry (Frozen sections)
    Sample
    Human Tissue sections (Brain section)
    Specification
    Brain section
    Fixative
    Paraformaldehyde
    Permeabilization
    Yes - 0.1x PBS plus 0.3xTriton x
    Blocking step
    Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 24°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.

    DR. Ruma Raha-Chowdhury

    Verified customer

    投稿 Sep 06 2010

    Western blot abreview for Anti-GBA antibody

    Good
    Abreviews
    Abreviews
    abreview image
    Application
    Western blot
    Sample
    Human Cell lysate - whole cell (HN10 cells)
    Loading amount
    10 µg
    Specification
    HN10 cells
    Treatment
    transfected with pcDNA-GBA
    Gel Running Conditions
    Reduced Denaturing (4-20%)
    Blocking step
    BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · 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 Jun 10 2009

    Question

    Product code: 55080
    Inquiry: I would like to have the MSDS, possibly an Italian MSDS, about the following product: Anti-GBA ab55080. Sincerely,

    Read More

    Abcam community

    Verified customer

    Asked on Sep 24 2012

    Answer

    Thank you for contacting us and sorry for the delay in getting back to you.

    We are not able to provide SDS documents in Italian but the English version can now be accessed from the datasheet of ab55080, or you will find it attached to this email.

    I hope this has been of help If you have any further requests, please do not hesitate to contact us again.

    Read More

    Abcam Scientific Support

    Answered on Sep 24 2012

    Question

    Thank you for prompt reply.
    Can I have the english version?
    Thank you again
    Best regards

    Read More

    Abcam community

    Verified customer

    Asked on Sep 17 2012

    Answer

    Thank you for your email.

    This antibody does not have any hazardous material in it so MSDS will not make any sense. For antibodies where hazardous material is used to preserve the solution the MSDS can be downloaded from online datasheet.

    I hope this information will be helpful. Should you have any question please do not hesitate to contact me.

    Read More

    Abcam Scientific Support

    Answered on Sep 17 2012

    Question

    Dear all,
    I would need of the italian version of material safety data sheet of your product Cat.n°ab55080.
    Is it avalaible?
    Thank you in advance
    Kind regards

    Read More

    Abcam community

    Verified customer

    Asked on Sep 17 2012

    Answer

    Thank you for contacting us.

    We unfortunately do not provide MSDS in Italian language. We have facility to provide MSDS in Chinese, Japanese, English, Spanish, French and German languages but not Italian.

    I hope this information is nevertheless helpful to you. Please do not hesitate to contact us if you need any more advice or information.

    Use our products? Submit an Abreview. Earn rewards!
    https://www.abcam.com/abreviews

    Read More

    Abcam Scientific Support

    Answered on Sep 17 2012

    Question

    Thank you for that information.  Actually, what we are looking for is a positive control, just to make sure that the Protein L antibody and then (on a separate experiment) the GCase antibody are working correctly.  Do you have any products that you would recommend as positive controls for the Protein L and GCase antibodies?

    Read More

    Abcam community

    Verified customer

    Asked on Apr 30 2012

    Answer

    Thank you for your response.

    A lysate ofPeptostreptococcus magnus,purified Protein L protein or recombinant Protein Lwould all be ideal positive controls for use withab63506.

    Fora positive control to use with our anti GBA antibody, ab55080. We have data on our website which shows the antibody to work withMCF-7 cell lysate and withHN10 cells. I would recommend one of those lysates for a positive control.

    I would like to encourage you to take advantage of our Abreview program. It takes just a few minutes to leave a review and you can collect Abpoints which you may redeem for Abcam products or Amazon gift cards while at the same time sharing information about the product with your colleagues worldwide. More information may be at:

    https://www.abcam.com/index.html?pageconfig=resource&rid=10332

    I hope that this information is helpful. Please let me know if you have any questions or there are other ways that Abcam may help you meet your research goals.

    Read More

    Abcam Scientific Support

    Answered on Apr 30 2012

    Question

    Hi,   We recently purchased your Anti-Protein L antibody (HRP) catalog number ab63506 and are interested in purchasing a loading control for this protein.  Could you please recommend one?

    Read More

    Abcam community

    Verified customer

    Asked on Apr 27 2012

    Answer

    Thank you for contacting us.

    When choosing a loading control it is best practice to choose housekeeping genes which will be present in your sample type. For example, in whole cell preparations beta Actin or GAPDH will be appropriate while in nuclear or mitochondrial fractions use Lamin B1 or COXIV respectively.

    These controls are excellent when you sample is eukaryotic. However, as Protein L will be from bacteria these housekeeping proteins may not be present. I have found however that GAPDH may be found in E.coli.

    I would recommend therefore that if your sample types are whole cell/membrane or cytoplasmic and are either bacterial (in the case of the Protein L antibody ab63506) or eukaryotic (as for use with anti GBA antibody ab55080) that you use our anti-GAPDH antibodies.

    We do have one anti-GAPDH product which is guaranteed to work with E.coli, Ab85760. Further information about this product may be found at the following page:

    https://www.abcam.com/GAPDH-antibody-HRP-ab85760.html


    I hope this information is helpful to you. Please do not hesitate to contact us if you need any more advice or information.

    Use our products? Submit an Abreview. Earn rewards!
    https://www.abcam.com/abreviews

    Read More

    Abcam Scientific Support

    Answered on Apr 27 2012

    Question

    I have just ordered a antibody from abcam, the mouse monoclonal to GBA (ab55080), that you refer as reactive with human and rat. I have tested it in both species in western blotting, and in human I can detect GBA, however, the bands were very faint. In rat, on the contrary, I could not detect any bands. Can you give me some tips? Above you will find some of the conditions that I used: - 80-100 ug protein/lane. (I have used human fibroblast and in the case of sat samples, I used total homogenates of several organs.) - blocking was done with 5% skimmed milk in TBS, for 1 h at room temperature - membrane was incubated overnight with the ab55080 antibody at 4ºC (used diluitions: 1:500; 1:1000 and 1:2000 in 5% milk in TBS) - incubation with secondary antibody: 1 h at room temperature - detection with AP.  

    Read More

    Abcam community

    Verified customer

    Asked on Dec 02 2011

    Answer

    Thank you for contacting us. I am sorry to hear that ab55080 is not providing satisfactory results in WB.  Having reviewed this case, I would like to offer some suggestions to help optimize your results.  I would also appreciate if you can confirm some further details: 1.)  When you say that the detection was done with AP, did it involve ECL reagents or was the detection colorometric?  We find ECL to be much more sensitive than colorometric detection, and we use ECL for our in house WB validation.  2.)  It may help to use a lower percentage of milk in your antibody diluent.  I typically recommend 0.1 - 1 % milk in the antibody diluent because higher concentrations can prevent the antibody from binding effectively to the protein of interest.  3.)  What secondary antibody was used and at what dilution?  For weak signal, it can sometimes help to use more secondary antibody.  Should the suggestions not improve the results, please do let me know. In the event that a product is not functioning in the species and applications cited on the product datasheet (and the problem has been reported within 6 months of purchase), we would be pleased to provide a free of charge replacement, credit note, or refund. I hope this information is helpful, and I thank you for your cooperation.  

    Read More

    Abcam Scientific Support

    Answered on Dec 05 2011

    Question

    The antibody does not work after freezing it. Antibody was bought last month and it worked very well. However when frozen aliquots were tried the antibody do not work at all. Different troubleshooting steps were tried with no success. The lysates used were human cancer cell line adenocarcinoma of cardiac. The secondary and other steps are OK because it worked fine before.

    Read More

    Abcam community

    Verified customer

    Asked on Sep 14 2011

    Answer

    Thank you for confirming these details and for your cooperation. The details provided enable us to closely monitor the quality of our products. I am sorry this product did not perform as stated on the datasheet and for the inconvenience this has caused. As requested, I have issued a free of charge replacement with the order number 949227. To check the status of the order please contact our Customer Service team and reference this number. Please note that this free of charge replacement vial is also covered by our Abpromise guarantee. Should you still be experiencing difficulties, or if you have any further questions, please do not hesitate to let us know. I wish you the best of luck with your research. PS: As discussed please try adding 50% Glycerol or 3% BSA before freezing these antibodies.

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    Abcam Scientific Support

    Answered on Sep 14 2011

    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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