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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| M | IH(P), ICC, WB | R | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MABN757 |
| Description | Anti-Beta-glucosidase 2 (GBA2) Antibody, clone 4A12 |
| Alternate Names |
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| Background Information | Non-lysosomal glucosylceramidase (EC 3.2.1.45; UniProt Q69ZF3; also known as Beta-glucocerebrosidase 2, Beta-glucosidase 2, Glucosylceramidase 2, NLGase) is encoded by the Gba2 (also known as Kiaa1605) gene (Gene ID 230101) in murine species. Glucosidases GBA1, GBA2, and GBA3 are structurally unrelated proteins with similar hydrolase activity that mediates glucosylceramide (GlcCer) degradation. GBA1 mediates GlcCer degradation in the lysosome lumen following its transport via the endosomal pathway. GBA2 is localized at the cytosolic surface of the ER and Golgi, with a predominant presence at the cis-Golgi in close proximity to the site of GlcCer synthesis and sphingomyelin conversion. GlcCer is also delivered to the ER through FAPP2-dependent transport and then flips to ER lumenal side by low specificity phospholipid flippases. GBA2 ER localization allows it to regulate GlcCer level at the cytosolic side of the ER and limit the amount of GlcCer from entering the ER lumenal side for higher order glycosphingolipid synthesis. Impaired GBA1 activity causes accumulation of GlcCer in lysosomes of tissue macrophages, causing liver and spleen enlargement and impairment of the central nervous system in patients with Gaucher disease. GBA2 activity is found downregulated in fibroblasts from a patient with type II Gaucher disease and fibroblasts from Gba1-deficient mice, suggesting a cross-talk between GBA1 and GBA2 that could affect the phenotype of Gaucher disease. GBA3 is a cytosolic Klotho-related protein whose physiological function is not yet known. |
| Product Information | |
|---|---|
| Format | Purified |
| Presentation | Purified rat IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Beta-glucosidase 2 (GBA2) Antibody, clone 4A12, Cat. No. MABN757, is a highly specific rat monoclonal antibody that targets beta-glucosidase 2 (GBA2) and has been tested in Immunocytochemistry, Immunohistochemistry (Paraffin), and Western Blotting. |
| Key Applications |
|
| Application Notes | Immunocytochemistry Analysis: A representative lot immunostained 4% paraformaldehyde-fixed, 0.5% Triton X-100-permeabilized, but not non-permeabilized, HEK293 transfectants expressing HA-tagged mouse beta-glucosidase 2 (GBA2-HA). GBA2-HA immunoreactivity was found associated with both Golgi and ER markers and greatly dependent on microtubule dynamics (Körschen, H.G., et al. (2013). J. Biol. Chem. 288(5):3381-3393). Immunocytochemistry Analysis: A representative lot detected beta-glucosidase 2 (GBA2) in isolated embryonic mouse hippocampal neurons, but not astrocytes (Körschen, H.G., et al. (2013). J. Biol. Chem. 288(5):3381-3393). Western Blotting Analysis: A representative lot detected HA-tagged mouse beta-glucosidase 2 exogenously expressed in HEK293 cells, as well endogenous beta-glucosidase 2 in testis tissue samples from wild-type, but not Gba2-knock mice (Körschen, H.G., et al. (2013). J. Biol. Chem. 288(5):3381-3393). |
| Biological Information | |
|---|---|
| Immunogen | Ovalbumin-conjugated linear peptide corresponding to a sequence from the N-terminal region of mouse beta-glucosidase 2 (GBA2). |
| Clone | 4A12 |
| Concentration | Please refer to lot specific datasheet. |
| Host | Rat |
| Specificity | Clone 4A12 detected the beta-glucosidase 2 (GBA2) target band in testis tissue sample from wild-type, but not Gba2-knock mice (Körschen, H.G., et al. (2013). J. Biol. Chem. 288(5):3381-3393). |
| Isotype | IgG1κ |
| Species Reactivity |
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| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Gene Symbol |
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| Purification Method | Protein G purified. |
| UniProt Number |
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| Molecular Weight | 103.3 kDa calculated. |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Immunohistochemistry in mouse brain tissue sections. Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected beta-glucosidase 2 (GBA2) in mouse brain tissue sections. |
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µL |