Overview

  • Product nameAnti-GBA antibody
    See all GBA primary antibodies
  • Description
    Mouse monoclonal to GBA
  • Tested applicationsSuitable for: WB, IHC-P, IHC-Fr, ICC/IFmore details
  • Species reactivity
    Reacts with: Rat, Human
  • Immunogen

    Recombinant fragment: SYFSEEGIGY NIIRVPMASC DFSIRTYTYA DTPDDFQLHN FSLPEEDTKL KIPLIHRALQ LAQRPVSLLA SPWTSPTWLK TNGAVNGKGS , corresponding to amino acids 146-236 of Human GBA

Applications

Our Abpromise guarantee covers the use of ab55080 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 Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 60 kDa.
IHC-P Use a concentration of 3 µg/ml.
IHC-Fr Use at an assay dependent concentration.
ICC/IF Use a concentration of 10 µg/ml.

Target

  • Involvement in diseaseDefects 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 similaritiesBelongs to the glycosyl hydrolase 30 family.
  • Cellular localizationLysosome membrane. Interaction with saposin-C promotes membrane association.
  • Information by UniProt
  • Database links
  • 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

Anti-GBA antibody images



  • Predicted band size : 60 kDa
    GBA antibody (ab55080) at 1ug/lane + MCF-7 cell lysate at 25ug/lane.
  • All lanes : Anti-GBA antibody (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
    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 is a courtesy of Anonymous Abreview

    See Abreview

  • ab55080 at 10 ug/ml staining GBA in human Hela cells by Immunocytochemistry/ Immunofluorescence.

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

References for Anti-GBA antibody (ab55080)

This product has been referenced in:
  • Yang C  et al. Celastrol increases glucocerebrosidase activity in Gaucher disease by modulating molecular chaperones. Proc Natl Acad Sci U S A 111:249-54 (2014). Read more (PubMed: 24351928) »
  • McNeill A  et al. Ambroxol improves lysosomal biochemistry in glucocerebrosidase mutation-linked Parkinson disease cells. Brain 137:1481-95 (2014). WB, ICC/IF ; Human . Read more (PubMed: 24574503) »

See all 5 Publications for this product

Product Wall

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 em...

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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.
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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.

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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...

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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 ...

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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...

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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 ha...

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Abcam guarantees this product to work in the species/application used in this Abreview.
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
Username

Dr. Ruma Raha-Chowdhury

Verified customer

Submitted Sep 07 2010

Abcam guarantees this product to work in the species/application used in this Abreview.
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
Username

Dr. Ruma Raha-Chowdhury

Verified customer

Submitted Sep 06 2010

Abcam guarantees this product to work in the species/application used in this Abreview.
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
Username

Abcam user community

Verified customer

Submitted Jun 10 2009

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"