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    biotin-gba-antibody-epr5142-ab201496.pdf

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RecombinantRabMAb

Recombinant Biotin Anti-GBA antibody [EPR5142] (ab201496)

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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Biotin Anti-GBA antibody [EPR5142] (ab201496)
  • Biotin Anti-GBA antibody [EPR5142] (ab201496)

Key features and details

  • Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
  • Biotin Rabbit monoclonal [EPR5142] to GBA
  • Suitable for: IHC-P
  • Reacts with: Human
  • Conjugation: Biotin

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Overview

  • Product name

    Biotin Anti-GBA antibody [EPR5142]
    See all GBA primary antibodies
  • Description

    Biotin Rabbit monoclonal [EPR5142] to GBA
  • Host species

    Rabbit
  • Conjugation

    Biotin
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide within Human GBA aa 50-150. The exact sequence is proprietary.

  • Positive control

    • IHC-P: Normal human thyroid tissue.
  • General notes

     

     

    This product is a recombinant monoclonal antibody, which offers several advantages including:

    • - High batch-to-batch consistency and reproducibility
    • - Improved sensitivity and specificity
    • - Long-term security of supply
    • - Animal-free production
    For more information see here.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

    Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.

    Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.

    We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.

    In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.

    We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.

    Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.

    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, as well as customer reviews and Q&As.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Avoid freeze / thaw cycle. Store In the Dark.
  • Storage buffer

    pH: 7.40
    Preservative: 0.02% Sodium azide
    Constituents: 30% Glycerol, 1% BSA, PBS
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Clonality

    Monoclonal
  • Clone number

    EPR5142
  • Isotype

    IgG
  • Research areas

    • 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
    • Pathways and Processes
    • Metabolic signaling pathways
    • Energy transfer pathways
    • Energy Metabolism
    • Metabolism
    • Types of disease
    • Neurodegenerative disease

Associated products

  • Alternative Versions

    • Anti-GBA antibody [EPR5142] (ab125065)
    • HRP Anti-GBA antibody [EPR5142] (ab200856)
    • Anti-GBA antibody [EPR5142] - BSA and Azide free (ab215261)
  • Isotype control

    • Biotin Rabbit IgG - Isotype Control (ab200208)
  • Recombinant Protein

    • Recombinant Mouse GBA protein (Tagged) (ab235724)

Applications

Our Abpromise guarantee covers the use of ab201496 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Target

  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the glycosyl hydrolase 30 family.
  • Cellular localization

    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
  • Database links

    • Entrez Gene: 2629 Human
    • Omim: 606463 Human
    • SwissProt: P04062 Human
    • Unigene: 282997 Human
    • Unigene: 719930 Human
    • Alternative names

      • 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

    Images

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Biotin Anti-GBA antibody [EPR5142] (ab201496)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Biotin Anti-GBA antibody [EPR5142] (ab201496)

      IHC image of GBA staining in a section of formalin-fixed paraffin-embedded normal human thyroid*, performed on a Leica Bond system using the standard protocol B. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab201496 at 1/50 dilution, for 15 mins at room temperature and detected using an HRP conjugated ABC system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

      The inset negative control image is taken from an identical assay without primary antibody.

      For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.

      *Tissue obtained from the Human Research Tissue Bank, supported by the NIHR Cambridge Biomedical Research Centre.

    • Biotin Anti-GBA antibody [EPR5142] (ab201496)
      Biotin Anti-GBA antibody [EPR5142] (ab201496)

    Protocols

    • Immunohistochemistry protocols

    Click here to view the general protocols

    Datasheets and documents

    • Datasheet

    Certificate of Compliance

    To download a Certificate of Compliance, please enter your Lot number below:

  • References (0)

    Publishing research using ab201496? Please let us know so that we can cite the reference in this datasheet.

    ab201496 has not yet been referenced specifically in any publications.

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