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Anti-GBA antibody (ab96256)

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  • SDS
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Abpromise

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Western blot - Anti-GBA antibody (ab96256)

    Key features and details

    • Rabbit polyclonal to GBA
    • Suitable for: WB
    • Reacts with: Human
    • Isotype: IgG

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    Recombinant Mouse GBA protein (Tagged) (ab235724)

    View more associated products

    Overview

    • Product name

      Anti-GBA antibody
      See all GBA primary antibodies
    • Description

      Rabbit polyclonal to GBA
    • Host species

      Rabbit
    • Tested applications

      Suitable for: WBmore details
    • Species reactivity

      Reacts with: Human
      Predicted to work with: Rat, Cow, Pig
    • Immunogen

      Recombinant fragment containing a sequence corresponding to a region within amino acids 65-354 of Human GBA (AAH03356).

    • Positive control

      • HeLa whole cell lysate; A431, H1299 and HepG2 cell lysates
    • General notes

      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. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
    • Storage buffer

      pH: 7.00
      Preservative: 0.025% Proclin 300
      Constituents: 79% PBS, 20% Glycerol (glycerin, glycerine)
    • Concentration information loading...
    • Purity

      Immunogen affinity purified
    • Clonality

      Polyclonal
    • 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
      • Metabolism
      • Types of disease
      • Cancer

    Associated products

    • 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)
    • Positive Controls

      • HeLa whole cell lysate (ab29545)
    • Recombinant Protein

      • Recombinant Mouse GBA protein (Tagged) (ab235724)

    Applications

    Our Abpromise guarantee covers the use of ab96256 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
    WB 1/500 - 1/3000. Predicted molecular weight: 60 kDa.

    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: 537087 Cow
      • Entrez Gene: 2629 Human
      • Entrez Gene: 449572 Pig
      • Entrez Gene: 684536 Rat
      • Omim: 606463 Human
      • SwissProt: Q2KHZ8 Cow
      • SwissProt: P04062 Human
      • SwissProt: Q70KH2 Pig
      • Unigene: 282997 Human
      • Unigene: 719930 Human
      see all
    • 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

    • Western blot - Anti-GBA antibody (ab96256)
      Western blot - Anti-GBA antibody (ab96256)
      Anti-GBA antibody (ab96256) at 1/1000 dilution + HeLa whole cell lysate at 30 µg

      Predicted band size: 60 kDa



      7.5% SDS-PAGE

    Protocols

    • Western blot protocols

    Click here to view the general protocols

    Datasheets and documents

    • Datasheet
    • SDS
  • References (0)

    Publishing research using ab96256? Please let us know so that we can cite the reference in this datasheet.

    ab96256 has not yet been referenced specifically in any publications.

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