MABN757

Anti-Beta-glucosidase 2 (GBA2) Antibody, clone 4A12

clone 4A12, from rat

• 同義詞: Non-lysosomal glucosylceramidase, Beta-glucocerebrosidase 2, Beta-glucosidase 2, Glucosylceramidase 2, NLGase
• 分類程式碼代碼: 12352203
• eCl@ss: 32160702

屬性

• 生物源: rat
• 品質等級: 100
• 抗體表格: purified antibody
• 抗體產品種類: primary antibodies
• 無性繁殖: 4A12, monoclonal
• 物種活性: mouse
• 技術: immunocytochemistry: suitable; immunohistochemistry: suitable (paraffin); western blot: suitable
• 同型: IgG1κ
• NCBI登錄號: NP_766280
• UniProt登錄號: Q69ZF3
• 運輸包裝: ambient
• 目標翻譯後修改: unmodified
• 基因資訊: mouse ... Gba2(230101)

描述

一般說明

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.

特異性

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).

免疫原

Ovalbumin-conjugated linear peptide corresponding to a sequence from the N-terminal region of mouse beta-glucosidase 2 (GBA2).

應用

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.

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).

Research Category
Neuroscience

品質

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.

標靶描述

103.3 kDa calculated.

外觀

Protein G purified.

Format: Purified

Purified rat IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

儲存和穩定性

Stable for 1 year at 2-8°C from date of receipt.

其他說明

Concentration: Please refer to lot specific datasheet.

免責聲明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

安全資訊

• 儲存類別代碼: 12 - Non Combustible Liquids
• 水污染物質分類（WGK）: WGK 1
• 閃點（°F）: Not applicable
• 閃點（°C）: Not applicable