概述

  • 产品名称
  • 描述
    小鼠单克隆抗体to GBA
  • 经测试应用
    适用于: WB, IHC-P, IHC-Fr, ICC/IFmore details
  • 种属反应性
    与反应: Rat, Human
  • 免疫原

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

应用

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.

应用 Ab评论 说明
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.

靶标

  • 疾病相关
    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.
  • 序列相似性
    Belongs to the glycosyl hydrolase 30 family.
  • 细胞定位
    Lysosome membrane. Interaction with saposin-C promotes membrane association.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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

Anti-GBA antibody 图像



  • Predicted band size : 60 kDa

    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 recognize GBA when GBA knockout samples were used, along with additional cross-reactive bands. 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 1/10000 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 1/10000 dilution for 1 hour at room temperature before imaging.

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



  • Predicted band size : 60 kDa
    GBA antibody (ab55080) at 1ug/lane + MCF-7 cell lysate at 25ug/lane.
  • GBA antibody (ab55080) used in immunohistochemistry at 3ug/ml on formalin fixed and paraffin embedded human breast cancer.
  • 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

Anti-GBA antibody (ab55080)参考文献

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...

Read More

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.
...

Read More

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 ne...

Read More

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...

Read More

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 ...

Read More

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...

Read More

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...

Read More
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

提交于 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

提交于 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

提交于 Jun 10 2009

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

注册