Anti-GBA抗体[2E2] (ab55080)
Key features and details
- Mouse monoclonal [2E2] to GBA
- Suitable for: WB, IHC-P, ICC/IF
- Knockout validated
- Reacts with: Human
- Isotype: IgG2a
概述
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产品名称
Anti-GBA抗体[2E2]
参阅全部 GBA 一抗 -
描述
小鼠单克隆抗体[2E2] to GBA -
宿主
Mouse -
经测试应用
适用于: WB, IHC-P, ICC/IFmore details -
种属反应性
与反应: Human -
免疫原
Recombinant fragment (GST-tag) corresponding to Human GBA aa 146-236.
Sequence:SYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKL KIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGS
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常规说明
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
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
存储溶液
pH: 7.4 -
Concentration information loading...
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纯度
Protein A purified -
克隆
单克隆 -
克隆编号
2E2 -
同种型
IgG2a -
轻链类型
kappa -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab55080于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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WB | (2) |
Use at an assay dependent concentration. Predicted molecular weight: 60 kDa.
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IHC-P |
Use at an assay dependent concentration.
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ICC/IF |
Use at an assay dependent concentration.
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说明 |
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WB
Use at an assay dependent concentration. Predicted molecular weight: 60 kDa. |
IHC-P
Use at an assay dependent concentration. |
ICC/IF
Use at an assay dependent concentration. |
靶标
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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
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数据库链接
- Entrez Gene: 2629 Human
- Omim: 606463 Human
- SwissProt: P04062 Human
- Unigene: 282997 Human
- Unigene: 719930 Human
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别名
- Acid beta glucosidase antibody
- Acid beta-glucosidase antibody
- Alglucerase antibody
see all
图片
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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.
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ab55080 at 10 ug/ml staining GBA in human Hela cells by Immunocytochemistry/ Immunofluorescence.
This image was generated using the ascites version of the product.
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GBA antibody (ab55080) at 1ug/lane + MCF-7 cell lysate at 25ug/lane.
This image was generated using the ascites version of the product.
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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.
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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 minuteThis image was generated using the ascites version of the product.
实验方案
数据表及文件
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SDS download
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Datasheet download
文献 (14)
ab55080 被引用在 14 文献中.
- 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